Lupus Essentials

Cover photo: Acute cutaneous lupus and alopecia in a 6-year old girl.

THIS IS A HOPE FOUNDATION BOOK Puiblished in 2021 by Hope Foundation "Warleigh", Lovell's Drive Welches, St. Michael, Barbados, BB11071 10 9 8 7 6 5 4 3 2 1 Text © Hope Foundation and Dr. Cindy Flower, MBBS, DM, FACP Pictures © Dr. Cindy Flower, MBBS, DM, FACP Design © Iconoclast Studios All rights reserved. This book is sold subject to the condition that it may not be reproduced, stored in a retrieval system or transmitted in any form or by any means, electronic, mechanical, photocopying, recording or otherwise without the publisher's expressed written persmission.

ISBN 978 976 96783 0 9 Publishing Manager: Shelley Weir Project Editor and Additional Text: Husani Weithers Design by Husani Weithers Production by Husani Weithers Printed in Barbados

ACKNOWLEDGEMENTS Respect and gratitude are owed to the following valued individuals: Ronald P Messner, the Director of the Division of Rheumatology, University of Minnesota (1993–1995) for the opportunity to train in rheumatology and for his instruction.

Charles Edwards, Head of the Department of Medicine (1994–2005), Queen Elizabeth Hospital, Barbados, whose vision for the department facilitated the establishment of several subspecialties including the Rheumatology service.

Anselm Hennis, Professor of Medicine and medical research scientist, for devoting his time and talent to ensuring the Barbados National Lupus Registry was brought to fruition.

Matthew H. Liang, Master of the American College of Rheumatology, for stellar mentorship, the demonstration of inclusivity and for the inspiration to tell our stories.

Michèle Callender, Consultant Dermatologist on the Medical Advisory Committee of Hope Foundation for her continued contribution to patient education.

To the “village” of medical and paramedical personnel who support the care of patients with lupus. Special tribute to the Internal Medicine Residents of the Queen Elizabeth Hospital who work tirelessly and sacrificially.

Hope Foundation volunteers, for their meaningful and beneficial support of the work of the organization.

The family and friends of the lupus patients who so willingly give their perspectives so that programs and activities can be structured holistically.

The lupus patients, for being our raison d’être and for all you have taught us.

ABOUT THE HOPE FOUNDATION Hope Foundation, established in Barbados in 1991 has become an influential participant in the healthcare of the nation through its work in patient advocacy and its major commitment to creating awareness of the lesser-known chronic diseases in Barbados. This has been accomplished by a group of dedicated and resolute volunteers united in a common vision of quality living for all.

The Foundation has benefitted significantly from inception by having outstanding leadership and a committed Medical Advisory Board. The overarching principle has been to offer guidance and expertise while being sensitive to the needs of patients in a participatory approach to the management of these chronic diseases.

Dedicated to the late George D. Nicholson DM (OXF), FRCP (LOND), FACP January 1 1937 – July 17 2016 Physician, Nephrologist, Educator, Researcher, Senior Medical Advisor to Hope Foundation

Professor George Nicholson was born in Guyana on January 1st, 1937. He attended high school at Queen’s College where he displayed academic and sporting excellence becoming Head boy and Captain of the football team. He went on to study Medicine at Oxford University where he continued his sporting pursuits on the rowing team.

In 1970, on return to the Caribbean he was appointed Lecturer in Medicine and Consultant Physician in Mona, Jamaica. Years later in 1979 he served as Consultant Nephrologist at the Queen Elizabeth Hospital in Barbados and made it his mission to improve healthcare by developing the Renal clinic and the Haemodialysis unit. He put together the team of medical professionals who conducted the first living related renal transplant in the Englishspeaking Caribbean. He was a founding member of the Barbados Kidney Association and the Caribbean Association of Urologists and Nephrologists.

Professor Nicholson was a stalwart of the University of the West Indies and lived a life of academic distinction. He served as Dean of the medical faculty 1992–1994, became Professor of Medicine in 1994 and Emeritus Professor in 2002.

Through his work in nephrology, he cared for hundreds of patients with lupus nephritis.

He developed a special interest and rare expertise in this disease overall. His renal clinic also became the first lupus clinic as it would accumulate all patients with lupus including those without renal disease.

In 1990, a 22-year-old Barbadian woman presented to Professor Nicholson’s clinic having returned from a visit to the USA during which she developed fevers, polyarthritis, a rash, sore throat and lymphadenopathy. Her accompanying medical report listed Adult Still’s disease as her diagnosis. After clinically reassessing the patient Professor Nicholson changed the diagnosis to SLE, noting in his concise summary that though sore throat and lymphadenopathy were common features of Still’s disease (Included as Yamaguchi minor

criteria 2 years later), he had witnessed those features to be prevalent in SLE. The final sentence elegantly written in that patient’s file is shown below and became a mantra —

“Young Black women of child-bearing age with Rheumatoidlike arthritis have SLE until proven otherwise.”

Weeks later that young woman developed the classic butterfly rash erasing any doubt.

Professor Nicholson’s charitable work with the Hope Foundation was an extension of the professional life he developed to educate and uplift the population. There were countless public lectures and talks to newly diagnosed patients with lupus. The patients respected and appreciated his contribution and were buoyed by his counsel.

Professor Nicholson’s wisdom was enviable and inspires us to continue his work to the highest standard — just as he taught and just as he would have expected.

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INTRODUCTION STEP 1: CASE ASCERTAINMENT STEP 2: DIAGNOSING LUPUS STEP 3: PRINCIPLES OF MANAGEMENT OF LUPUS STEP 4: LIFE & LUPUS REFERENCES APPENDICES ABOUT THE AUTHORS

CONTENTS

LUPUS ESSENTIALS

INTRODUCTION

Lupus (systemic lupus erythematosus or SLE), a disease with protean manifestations, is unpredictably different in each individual.

It is a chronic systemic autoimmune disease with periods of illness (flares) and periods of wellness (remission), caused by a complex interplay of genetic, hormonal, and environmental factors. It occurs most commonly in Black women of childbearing age though anyone can be affected.

Cases of lupus often cluster in poorer communities where international guidelines for its optimal management cannot be strictly applied. In many such

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community. It sets forth a path of continuous lifelong learning and a journey that sees the convergence of real life and Lupus Essentials highlights crucial science. information on the diagnosis and management of lupus especially in populations where a variety of resource-constraints exist. In decades of caring for persons with lupus in Barbados, the authors have attempted to balance current assessment and treatment options with the social interventions necessitated by the hardships and realities of poverty. communities the disease is often diagnosed and managed by nonspecialists.

The complexity of Lupus frequently humbles and challenges the medical

STEP 1

CASE ASCERTAINMENT In Barbados, women are 14 times more likely to develop lupus than men and the disease is three times more common in persons of African origin compared to Caucasian populations. Lupus predominates in the 15–44 year age group. Thus, the typical lupus patient is a young Black woman of child-bearing age. However, ANYONE can develop lupus. The disease has been seen in boys and girls as young as 4 years of age and elderly individuals first diagnosed over the age of 70. It is a disease seen globally in all races.

Lupus is a great mimic, a heterogenous disease involving multiple body organs with a vast array of presenting complaints, clinical features and varied course. It can occur suddenly and severely resulting in disability or death, though most commonly it evolves over time with periods of activity and the risk of cumulative damage from the disease or the drugs used to treat it.

The most common symptoms which should prompt an individual to present for medical attention are-: Painful joints with swelling and early morning stiffness Rashes especially on the cheeks, nose, ears, scalp, elbows

Chest pain on deep breathing Hair loss Sores in the mouth Unexplained fever

Sun-sensitivity with rashes

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Step 1: Case Ascertainment

Some of the features which should make a doctor think of lupus as a possibility are-:

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Patient presenting with aforementioned symptoms, occurring concurrently or sequentially over time

Patient, especially a young female, with proteinuria and/or impaired renal function

Patient, especially a young female, with unexplained organ or system disorder

Patient with evidence of a condition affecting multiple systems of the body

Young patient with unprovoked thrombosis

Any patient with an obscure diagnosis or a diagnostic dilemma

Bonus Tip Beware of the vague complaint: "I feel sick" On the journey to diagnosis of lupus or sometimes as a flare is unfolding, a patient may report vague symptoms or simply articulate "I feel sick". This patient, even with a seemingly unremarkable physical examination, may in fact be extremely ill and at risk of sudden, severe deterioration with adverse outcome. Subtle laboratory abnormalities that may be seen include anemia of chronic inflammation, lymphopenia, and hypoalbuminemia (as an acute phase response). These are red flags. The doctor must be sensitive to this issue and remain vigilant — vague symptoms may be the subclinical stage of an impending explosion of disease.

The role of professional and public awareness of lupus in case ascertainment To facilitate early case ascertainment, medical professionals and the general public need to be made aware or reminded of the existence of lupus and the broad spectrum of what it can do.

This is accomplished through education in medical school and and continuous medical education programs. The public is reached through the work of lupus charities and support groups assisted by the media.

A patient with lupus may recognize the development of the disease in a relative — for example by their familiarity with the appearance of discoid lesions. Surgeons have encountered and suspected lupus in their patients with acalculous cholecystitis or with unexplained pancreatitis — both seen as initial presentations of lupus.

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STEP 2

DIAGNOSING LUPUS

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The diagnosis of lupus is based on history and physical examination supported by laboratory abnormalities. The disease can develop over days to weeks or evolve more slowly over months and even years.

Cases with classic features are readily diagnosed while others require more in-depth investigation to exclude other possible conditions. Complex cases may require a trained specialist while some cases are best seen by a physician experienced in the wide range of lupus presentations who has an almost intuitive recognition of the disease.

A hallmark of lupus is the development for antibodies to self-antigens, some of which can be measured. The classic autoantibody test — the antinuclear antibody (ANA) — is positive in over 95% of cases of lupus but this sensitive test is not specific to the disease.

A positive ANA may be seen in a host of rheumatic and non-rheumatic conditions and even in some healthy individuals. The most specific autoantibody test in lupus — the anti-Smith antibody — is positive in only 25% of cases and as such cannot be used as a screening test for the disease. Unlike a chronic disease such as diabetes mellitus where the lab abnormality is key to the diagnosis, there is no sidestepping the sometimes tedious work of the medical history, exam and clinical reasoning in working towards a diagnosis of lupus.

Step 2: Diagnosing Lupus

Laboratory abnormalities which when present (and accurate) are supportive of a diagnosis of lupus-:

Anemia — due to chronic inflammation (more common) or haemolysis Positive direct Coombs’s test Leukopenia/lymphopenia Thrombocytopenia Positive ANA — highly sensitive test positive in 95% of lupus cases, not specific Positive Anti-dsDNA — specific test, positive in 50% of lupus cases, not sensitive

Positive Anti-Smith — highly specific test, positive in 25% lupus cases, not sensitive Positive antiphospholipid antibodies Low complements Spot urinary protein creatinine ratio >0.5

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Step 2: Diagnosing Lupus

On ward rounds, the late Professor Nicholson was taken to the bedside of a young Rastafarian woman who was admitted with hypertension, proteinuria and renal failure. With lupus being a strong possibility — conditions like primary vasculitis or infection-associated glomerulonephritis were less common — it was reported that the patient had no joint pain, no chest pain, no oral ulcers, no hair loss, no rashes. After a short pause Professor Nicholson approached the patient asking, “Do you have any bald patches?”— she unhesitatingly said “yes,” flipping her long dreadlocs to reveal an extensive discoid lesion with scarring alopecia. The embarrassing lesson here is that doctors need to ensure that they are understood, use probing open-ended questions and sometimes ask the same question in different ways. The physical examination must also be consistently meticulous.

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Joints

COMMON CLINICAL FEATURES OF LUPUS

The most common feature of lupus is an inflammatory polyarthritis that mimics rheumatoid arthritis. The small joints of the hand and the knees are typically affected with evidence of synovitis and joint effusions and over 30 minutes of early morning stiffness. In some patients with inflamed joints the physical findings may be subtle.

Fig 3.1: Swelling of the knees with effusions distending the suprapatellar region

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Step 2: Diagnosing Lupus

Fig 3.2: Diffuse swelling of the small joints of the hands

Fig 3.3: Jaccoud’s arthropathy — a chronic, deforming non-erosive arthropathy of the hands that mimics rheumatoid arthritis. It is initially passively correctable but in some cases the deformities become fixed

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swan neck

Z thumb

Step 2: Diagnosing Lupus

The integumentary system and oral mucosa The integumentary system consisting of the skin, hair and nails is frequently affected in lupus.

Patients with potential SLE may use makeup, wigs, weaves, headscarves, and nail art that obscure the physical findings. The doctor must be a good detective and look beyond these adornments to perform a complete examination. distinct sparing of the nasolabial folds in this severe facial rash periorbital edema may occur as part of acute cutaneous lupus in the absence of proteinuria

The Butterfly rash — also called acute cutaneous lupus — is non-scarring and has a characteristic distribution around the nose and cheeks with relative sparing of the nasolabial folds; it is lupus specific. The appearance may vary from hyperpigmented to hypopigmented. Erythematous lesions may be evident in fairer-skinned patients.

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Step 2: Diagnosing Lupus

Fig 3.4: This patient presented with retinal vasculitis, the initially obscured hyperpigmented malar rash was made clear after her makeup was removed.

Fig 3.5: Hyperpigmented malar rash; part of a first presentation of lupus.

Fig 3.6: The cause of this patient’s pancytopenia was revealed when this hypopigmented malar rash developed.

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Step 2: Diagnosing Lupus

Fig 3.7: Erythematous plaques are evident in fair-skinned women.

Fig 3.8: Before the malar rash developed this gentleman was being investigated for possible leukemia/lymphoma because of his febrile neutropenia and pericardial effusion.

Fig 3.9: A more subtle but significant malar rash — a slightly erythematous plaque on the right cheek and a less well-defined lesion on the left cheek.

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Step 2: Diagnosing Lupus

Discoid lupus, also called chronic cutaneous lupus, is characterized by well-defined photosensitive inflammatory plaques that frequently and quickly evolve into atrophic depigmented scars.

It may occur independently of systemic lupus erythematosus. The most characteristic locations are the conchal fossa of the external ear, the scalp, and the extensor elbows.

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Interestingly it may also occur in a "Butterfly" distribution.

The chronic inflammation and depigmentation predispose to the formation of secondary squamous cell carcinoma.

Fig 3.10: Extensive discoid lesions in a characteristic area — the extensor aspect of the elbows.

The central depigmentation and surrounding hyperpigmentation are characteristic of healed lesions.

early coin-shaped lesions for which the condition is named

Fig 3.11: Discoid lesions on the left elbow.

Step 2: Diagnosing Lupus

Fig 3.14: When this elderly lady presented to her GP with new-onset polyarthritis, a thorough physical exam revealed discoid scalp and external ear lesions with scarring alopecia and led to the diagnosis of lupus

Fig 3.12: Discoid lesions in the palm — areas of erythema and scale are seen in active lesions.

Fig 3.13: Discoid lesion on the lower lip — hyperpigmented raised border with central erythema. The risk of secondary malignant transformation is thought to be heightened in this location.

Fig 3.15: Discoid lesions in conchal fossa of the right ear with additional lesions on the side of the face and neck.

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Step 2: Diagnosing Lupus

Fig 3.16: Discoid rash in a “butterfly” distribution

Fig 3.17: Severe discoid lupus with scarring alopecia, note lip involvement

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Fig 3.18: “Lupus hair”straightening of the hair texture and non-scarring alopecia

Fig 3.19: Atrophic depressed discoid scars due to accompanying subcutaneous fat atrophy

Step 2: Diagnosing Lupus

Subacute cutaneous lupus

Fig 3.20: Scarred depigmented discoid lesions with secondary squamous cell carcinoma in the right hand of this patient

Fig 3.21: Extensive discoid lesions on one forearm while the other has only a few small lesions. This illustrates photoexacerbation of discoid lupus on the arm exposed to frequent sunlight in this driver

Subacute cutaneous lupus is a non-scarring photosensitive rash that is more common in Caucasian patients hence has not been commonly seen in Barbados.

Fig 3.22: Lupus patient with subacute cutaneous lupus occurring days after spending a day at the beach. The rash spared areas of the back covered by the swimsuit.

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Step 2: Diagnosing Lupus

Nails

Fig 3.23: Blue-black nail dyschromia is described in Black patients with lupus — it is benign and possibly due to melanocyte hyperplasia or activation.

This young woman was initially too distressed by the appearance of her nails to focus on the seriousness of her lupus nephritis. Fortunately, her perspective shifted after being complimented by her peers on her “cool manicure”.

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Fig 3.24: Acquired transverse leukonychia (Mee’s lines) may develop due to abnormality of the nail plate coinciding with a serious lupus flare this patient experienced weeks earlier.

Step 2: Diagnosing Lupus

Oral ulceration

Raynaud's A frequent question asked is whether Raynaud's is seen in our tropical environment — the answer is yes, infrequently and not usually severe in our lupus patients.

Fig 3.25: The classic oral ulcer in lupus requires an active search. It is located on the hard palate and is painless- this patient was not aware of its presence. On rare occasion these ulcers cause palatal perforation.

Fig 3.26: Attack of Raynaud’s in an air-conditioned medical office. The ischaemic phase with a demarcated area of blanching is evident in 3rd/4th fingertips.

Fig 3.27: Rare occurrence of gangrene of the tip of the left 5th finger in a teenager after severe Raynaud’s.

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Step 2: Diagnosing Lupus

Common cardiopulmonary complications Pericarditis is suggested by a history of retrosternal chest pain exacerbated by lying flat and ameliorated by leaning forward. Large pericardial effusions with risk of cardiac tamponade may occur with late presentation. Rarely, constrictive pericarditis develops.

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Pleurisy is suggested by chest pain on inspiration and may be unilateral.

The pain may be felt in the shoulders, upper abdomen or back which sometimes causes misdiagnosis.

Small pleural effusions may develop bilaterally but large unilateral effusions requiring thoracocentesis are also seen.

Fig 3.28: Large pericardial effusion in young woman with SLE.

Fig 3.29: Large pleural effusion with whiteout of the left hemithorax in young woman with SLE. Younger patients may be remarkably tolerant of these complications- this patient reported only a oneweek history of exertional “discomfort”.

Step 2: Diagnosing Lupus

Neuropsychiatric lupus Neuropsychiatric lupus (NPSLE) refers to a broad array of neurologic disease where the primary process is due to lupus autoimmunity. The diagnosis is based on clinical judgement as there are no pathognomonic clinical, laboratory or imaging features. NPSLE is an early complication with most cases developing within the first 2 years of lupus diagnosis, predominantly involving the central nervous system — with fewer cases involving the peripheral nervous system — and it typically occurs in the setting of features of high global lupus activity. These facts are

used to support the clinical attribution of complications to NPSLE. The most common NPSLE complications in our setting in order of decreasing frequency are psychosis, stroke, and seizures. In our experience, even in lupus patients on high dose steroids, psychosis is much more likely to be due to lupus than to the steroids. My mentor, the late Professor Nicholson and I have never diagnosed steroidinduced psychosis in a lupus patient.

Imaging of the brain in patients with NPSLE is frequently

normal. Some patients are found to have white matter hyperintensity lesions on Magnetic Resonance Imaging as in the case below.

The white matter hyperintensity lesions shown here correlate with small vessel disease and can be seen in NPSLE but are not pathognomonic.

Fig 3.30: MRI (T2 weighted images) of the brain of a teenage boy diagnosed with acute confusional state due to NPSLE.

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Step 2: Diagnosing Lupus

Lupus patients may have an array of central nervous system complications unrelated to lupus autoimmunity. Imaging studies are useful in diagnosing some of these. The following 3 examples are of different non-NPSLE complications evident in 3 different lupus patients who each developed seizures.

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Fig 3.31: CAT scan of the brain-young woman with a late presentation of lupus nephritis in end-stage renal failure developed a large left intracerebral bleed with surrounding oedema

Fig 3.32: CAT scan of the brainTeenager on treatment for lupus nephritis developed posterior reversible encephalopathy syndrome (PRES) — note the hypoattenuation predominantly in the occipital area.

Fig 3.33: CAT scan of the brain Multiple cerebral micro-abcesses in the setting of staphylococcus aureus septicemia in a patient with lupus nephritis on mycophenolate mofetil. The primary site of staph aureus was an infected peripheral intravenous line demonstrating that simple preventable issues can result in catastrophic consequences.

Step 2: Diagnosing Lupus

Lupus nephritis Lupus nephritis is more prevalent and severe in Black patients. It is the complication of lupus that in our setting has the highest morbidity and mortality.

Unlike complications detailed previously, it is silent in the early stages. The earliest detection comes from screening with a test as simple as a urine dipstick which the patient can be taught to do on their own at home.

Since lupus nephritis typically develops within 3 years of lupus diagnosis, we recommend all newly diagnosed lupus patients perform monthly at-home

urinalysis for protein. A negative dipstick test is usually accurate for excluding proteinuria. Any positive results require official quantification e.g., with spot urinary protein creatinine ratio.

“Young Black women of childbearing age with proteinuria have lupus until proven otherwise.”

If the mantra were broadened to “Individuals with proteinuria in the absence of diabetes and/or primary hypertension have lupus until proven otherwise” more patients with

lupus nephritis would be diagnosed earlier before the development of florid signs of renal disease including renal failure.

Lupus nephritis is such a potentially perilous complication often an immediate or early decision must be taken to initiate disease-directed therapy — in our setting in the absence of serologic or histologic data which are often delayed or unavailable.

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Step 2: Diagnosing Lupus

Clinical utility of classification criteria for lupus Fig 3.34: Pedal oedema due to nephrotic syndrome in lupus nephritis- a late presentation in a newly diagnosed teenager with lupus. The skin shows the depigmented but non-scarring lesions of healed bullous lupus.

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Fig 3.35: The same patient during the phase of active bullous lupus

Classification criteria like the 3 listed (ACR/SLICC/EULAR-ACR — Appendix A) are useful for study purposes but are expressly not diagnostic criteria. The clinical usefulness of these criteria is that they do identify definite cases of lupus, however because lupus is such a heterogenous disease there will be clinical cases of lupus who do not satisfy the classification criteria.

Doctors must be clear on this perspective to avoid misdiagnosing and mismanaging cases of lupus who have clinical features not

Step 2: Diagnosing Lupus

contained in classification criteria. For example, there are 19 neuropsychiatric syndromes well-described to be a part of lupus — only 2–5 of those are part of the various classification criteria. The scope of the disease is too vast to be captured by any classification tool and the art of diagnosis cannot be usurped.

Bonus Tip In lupus a clinical complication may be resolving while another is unfolding.

Putting it all together The most important part of diagnosing lupus is simply thinking about it.

Lupus may have a very classic presentation allowing for an easy diagnosis e.g., a patient with a butterfly rash, polyarthritis, pleurisy, and leucopenia.

A patient may present with a clinical scenario where lupus enters the differential diagnosis and further investigation, or assessment is needed e.g. a patient with isolated nephrotic syndrome A report of a negative ANA does not rule out lupus where the clinical suspicion is high, and a positive ANA is not sufficient for a diagnosis of lupus where the clinical picture is not in keeping with the disease.

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Step 2: Diagnosing Lupus

After a case of lupus is diagnosed it is important to identify factors that may negatively impact on patient outcomes such as low socioeconomic status, low education attainment, low selfefficacy, lack of a family or social network, and psychological issues such as depression and anxiety.

Management of patients with these confounders goes far beyond the pharmaceutical prescription to involve referrals to social workers, psychologists and a host of social support services and charities.

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Fig 3.36: Sample prescription to reduce risk of renal flare in a 24-year-old who has controlled lupus nephritis and is the working mother of a toddler

PRINCIPLES OF MANAGEMENT OF LUPUS

STEP 3

Lupus patients require close long-term follow-up with the frequency of assessment dictated by the severity of the disease. Management involves use of background medication, system-specific medication, treatment of comorbidities, infection prevention and attention to quality-of-life issues.

Background medication in lupus consists of: Hydroxychloroquine 5mg/kg daily (ideal body weight to a max. of 400mg daily) which has a therapeutic effect for joint and skin disease as well as being antithrombotic, positively affecting lipid profiles and reducing the risk of development of Type 2 Diabetes Mellitus.

Vitamin D3 400 units daily thought to ameliorate inflammatory markers Sunblock/sun-precautions

These interventions are simple and safe enough to be prescribed by the non-specialist.

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Step 3: Principles of Management of Lupus

Before the appropriate Example 1: Renal impairment in a lupus therapeutic strategy is patient may be due to active nephritis, or renal sclerosis from past nephritis, or may be launched for a clinical a side effect of medication taken like NSAIDs complication, one first for joint pain or may be due to an intercurrent has to be certain there is illness like leptospirosis or sepsis, or a concurrent disease like diabetes mellitus or correct attribution. A clinical hypertension. complication in a lupus patient may be due to: 1. Active lupus Example 3: Skin lesions in a lupus patient may be due to active cutaneous lupus, may be a resolved rash such as 2. Secondary damage discoid scars, may be an allergic reaction to medication*, from lupus may be an intercurrent illness such as herpes zoster or 3. A side effect of scabies** or may be a concurrent disease such as lichen planus. therapy' 4. Intercurrent illness 5. Concurrent disease

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Example 5: Anemia in a lupus patient may be due to autoimmune haemolytic anemia — a manifestation of active lupus, may be due to end stage renal failure from lupus nephritis, may be drug-induced, may be an intercurrent disorder causing iron deficiency e.g. menorrhagia associated with fibroids, may be a concurrent illness like pernicious anemia.

Example 2: A pericardial effusion in a lupus patient may be due to lupus pericarditis, may occur in the setting of uremic pericarditis from end stage renal failure in lupus nephritis, may be a side effect of medication such as minoxidil for severe hypertension, may be infectious or may occur from a concurrent illness such as hypothyroidism.

Example 4: Joint pain in a lupus patient may be due to active lupus synovitis, or avascular necrosis of bone, or may be a side effect of drugs such as statins or Accutaneused for rashes. It may be due to an intercurrent illness such as gonococcal arthritis or a concurrent condition such stress-induced somatic sensory disorder.

Step 3: Principles of Management of Lupus

**Classic discoid lesions on the forearm of a patient who developed another rash on the back and buttocks which was fiercely pruritic at night

Several scattered hyperpigmented papules with areas of hyperkeratosis representing scabies with areas of excoriation from scratching

*Purpuric circinate annular plaques — a drug eruption in a patient with lupus nephritis

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Step 3: Principles of Management of Lupus

Once a clinical complication is assessed as being a consequence of lupus, treatment moves beyond supportive/ancillary care to disease-directed therapy.

See Appendix B for an example of a strategy using severity stratification to address the more common complications.

While sound clinical reasoning is an enduring feature of the practice of medicine, treatment guidelines all have an expiration date.

WHY THE FIRST 3 YEARS ARE CRITICAL The first 3 years after the diagnosis of lupus are critical for the following reasons: Serious complications of the disease notably nephritis and neuropsychiatric lupus are early complications tending to unfold within the first 2–3 years. Younger patients have more severe lupus nephritis than older patients.

Medication non-adherence is highest in patients with shorter disease duration and in younger patients.

Hence there is a set up for a perfect storm.

An extremely ill patient, at risk of omitting medication when it is most needed.

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Step 3: Principles of Management of Lupus

Interventions to address medication non-adherence are as complex as the issue itself.

Although there are potent social, economic, and cultural causes, often overlooked “medical” causes of medication nonadherence are cognitive dysfunction and depression.

To further complicate matters, cognitive dysfunction and depression may be neuropsychiatric manifestations of the disease. In other words —

We have seen patients decline reasonable and safe medical interventions or therapy, sometimes citing religious reasons only to eventually realize that this behavior was a prodrome to lupus psychosis.

Medication nonadherence may be both the cause AND the consequence of a lupus flare.

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STEP 4

LIFE AND LUPUS Social, environmental, psychological and economic conditions impact the health of patients with lupus and the work of support groups can buttress the patients, their loved ones, and the medical system that serves them. The areas deemed to be of particular relevance involve the intersection of lupus with the activity of bringing up children, with navigation of life’s emotional stressors and with the struggle of overcoming poverty.

Lupus and Parenting

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Beyond lupus and pregnancy is a more complete narrative — lupus and parenting.

Pregnancy is an event with well-defined medical recommendations to prevent, to terminate or to maximize safe completion. There are many excellent resources addressing this including the website LupusPregnancy.

Parenting is a responsibility, a biopsychosocial construct with long-term consequences. If an impoverished young woman with lupus has an unplanned entry into motherhood, it further exacerbates her vulnerable social situation with a higher risk of adverse outcome for the disease.

Social issues aside, motherhood also impacts therapeutic decisions in ways that are

complex and the medical team has to be sensitive and understanding of the patient’s wishes.

A young woman in the first trimester of her first pregnancy presents with an acute onset of lower limb weakness progressing to paraplegia with bowel and bladder dysfunction over a matter of hours. The Magnetic Resonance Imaging confirms the clinical diagnosis of longitudinally extensive transverse myelitis with almost complete involvement of the spinal cord.

Step 4: Life and Lupus

The patient declines aggressive treatment options that include use of cyclophosphamide and termination of pregnancy and is reluctant to use any medication except those categorized as pregnancy compatible.

Her entire focus is on preserving the pregnancy even at the risk of being a paraplegic mother.

Another lupus patient with a similar presentation, in the first trimester of her second pregnancy, is anxious to have the most aggressive treatment to maximize her chances of recovery. This patient reports that as a single mother her priority is to remain a fit parent to the young child she has at home.

T2 weighted images showing increased signal in the cervical and thoracic cord

Fig 3.37: MRI of the patient's cervical spine

Fig 3.38: MRI of the patient's throacic spine

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Step 4: Life and Lupus

Psychosocial Stress

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One of the most frustrating issues in assessment of patients with lupus is the impact of emotional stress on the development or amplification of somatic symptoms. This is an issue that some patients are apt to find puzzling or insulting and the doctor may be uncomfortable initiating such a discussion. Quite commonly, what follows are a series of unnecessary investigations, medication and even procedures accompanied by distressing persistence of the symptoms.

Medical professionals and patients need to accept that

emotional stress can cause a somatic sensory disorder. It is a simple human phenomenon and should not be infused with any derogatory connotation.

Additionally, futile care has inherent risks. The best therapy in an insightful patient is reassurance combined with referral to psychology and psychiatry.

Step 4: Life and Lupus

Example case A 60-year-old woman with lupus was referred to the rheumatology clinic for assessment of chronic malaise with atypical chest pain. Her physical exam was remarkable for scarring alopecia only and all basic investigations were normal. Her file revealed the following past medical history-: • 5 unremarkable upper endoscopies • Innumerous ECG’s, Holter monitors, cardiac echocardiograms, CT pulmonary angiography, Chest Xrays and one coronary angiogram — all normal • Surgical consultations with normal abdominal imaging and one occasion in which the patient was prepared for exploratory surgery before it was deemed unnecessary The patient was reassured that there was no evidence of lupus activity, or any concurrent disease and a frank discussion was initiated on the role of stress in inducing physical symptoms. The patient admitted to having mental health issues surrounding domestic stress for which she had never sought professional help and was then appropriately referred.

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Step 4: Life and Lupus

Lupus and Poverty

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Poverty is considered the single largest determinant of health in general and profoundly affects lupus outcome. The needs of impoverished patients go far beyond medical issues. It can seem insurmountable to address all the social requirements such as employment, housing, childcare, and general financial aid. However, their adverse impact on prognosis is so profound that managing these issues is all part of holistic care.

The following excerpt from the Barbados Today newspaper (June 6, 2021) illustrates some of the challenges voiced by patients:

"CD, who was recently diagnosed with lupus, is homeless and crying out for help. The struggling, unemployed young woman who is finding it hard to make ends meet, is asking for assistance with a housing solution for her and her five-year-old son and eleven-year-old daughter. Last Friday night, CD was thrown out of the Black Rock, St Michael house where she was living with her aunt and grandmother. And though she is still struggling to come to grips with having to learn to cope with the emotional and physical pain of living with the autoimmune disease, the young mother is asking for the opportunity to work so she would be able to provide for her children. “My situation right now is to find a job; it is to find somewhere to live because where I am now is only a temporary situation. I need somewhere to live for me, and my two kids and I really want work. I can cook, I can bake, so any work along those lines would be perfect for me,” she said."

In Barbados, where 98% of lupus patients are of Africanorigin, lupus patients of lower socioeconomic status appear to have a more severe disease phenotype from the onset with more major organ involvement and lupus nephritis that those deemed to be of a higher socioeconomic status. It is possible that socioeconomic determinants affect clinical features of lupus and that poorer patients are less likely to have what we term “lupus lite”. This area requires further study.

Step 4: Life and Lupus

The impact of the work of lupus support groups While Hope Foundation does not have the financial resources to provide welfare support, we do maintain active relationships with service clubs, health agencies and the business community to endorse the assistance needed by dire cases such as CD. The goodwill and reputation built over the years is invaluable in this regard.

One of Hope Foundation’s most effective educational interventions has been the Annual Seminar for Newly Diagnosed Patients. It brings doctors and patients together in an informal setting that: Improves communication between the two parties Instils confidence in patients to ask questions Promotes self-awareness to identify evolving problems more easily Includes relatives and friends so that they too have a better understanding of the disease and therefore lend appropriate support

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Step 4: Life and Lupus

Hope Foundation, like other lupus support groups, gives patients a voice and has reported five (5) important assertions that patients have expressed to bring clarity to the medical team: 1. Lupus is not just a disease — it is a person. A person with a domestic and community footprint. In addition to the broad spectrum of disease complications, there is a difference among persons in how lupus is experienced.

2. There are times when the gravity of what the doctor is saying during the consultation is too difficult to grapple with in the moment — have patience, continue the conversation and remember to listen.

3. The clinic or hospital is often unfamiliar territory especially as most lupus patients are young and have been previously well. Ensure the navigation of the facility is clarified and does not add an additional layer of stress.

4. While empowering patients to participate in their own care, it is understood that the doctor must maintain autonomy to make the best clinical decisions.

5. It is vital for the newly diagnosed lupus patient to be formally referred to the Lupus support group. The services and benefits complement what is provided in the clinical setting. Never underestimate the impact of the lupus support group.

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Step 4: Life and Lupus

Example case A 45-year-old woman with a 13-year history of lupus attended an annual seminar for newly diagnosed lupus patients hosted by Hope Foundation one Sunday morning, She was burdened by a secret.

During the coffee break she found the courage to confide in other patients that 3 months earlier she had developed a painful swelling on the back of her head and was fearful to present for medical assessment. They formed an impromptu circle of support and retreated to the Ladies Room where the woman removed her headscarf, a wig, and a rudimentary dressing to reveal a necrotic mass on the occiput over an area of extensive discoid scarring — later biopsy-proven squamous cell carcinoma (see image)

Fig 3.39: Squamous cell carcinoma in an area of depigmented discoid scars

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REFERENCES

1. Flower C, Hennis AJ, Hambleton IR, Nicholson GD. Lupus nephritis in an AfroCaribbean population-: renal indices and clinical outcomes. Lupus 2006; 15:689–694.

2. Flower C, Hennis AJ, Liang MH. Report of an International Symposium on narrowing the Gap in the Treatment and Study of SLE worldwide: Minimum best practices in the management and monitoring of moderate to severe SLE and improving outcomes in constrained environments. Curr Rev Musculoskelet Med 2011; 4:105–112.

3. Flower C, Nicholson GD. Management of Lupus nephritis in constrained environments. Current Medical Literature — Rheumatology 2011; 30(3):73–80 4. Flower C, Hennis AJ, Hambleton IR, Nicholson GD, Liang MH. Systemic Lupus Erythematosus in an Afro-Caribbean population: incidence, clinical manifestations and survival in the Barbados National Lupus Registry. Arthritis Care and Research 2012; 64: 1151–1158 5. Flower C, Gaskin D, Bhamjee S, Bynoe Z. High-risk variants of cutaneous squamous cell carcinoma in patients with discoid lupus erythematosus: a case series. Lupus 2013; 22: 736–9.

6. Flower C. Lupus Psychosis in Black Caribbean population. Abstract Lupus Sci Med 2016 7. Flower C, Hambleton I, Campbell M. The effect of psychosocial and neuropsychiatric factors on medication adherence in a cohort of women with systemic lupus erythematosus. J Clin Rheumatol.

2016; 22: 411–417.

8. Flower C, Hambleton I, Corbin D, Marquez S, Edghill R. The spectrum of neuropsychiatric lupus in a Black Caribbean population — a report of the Barbados Lupus Registry. Lupus 2017; 26:1034–1041 9. Flower C. Socioeconomic determinants and SLE severity in black patients in Barbados. Lupus Sci Med 2018;5(Suppl 2):A1–A81 10. Flower C. Assessment of SLE activity for acute clinical decision-making: Use of a color-coded threatlevel approach for non-rheumatologists. Lupus Sci Med 2021; Feb;8(1):e000484.

11. Flower C. The therapeutic skill of reassurance — a case report of high utilization of medical services for distressing but benign somatic complaints. BAMP Bulletin Sept–Oct 2013, pg 24

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APPENDIX B

Source: Lupus Sci Med 2021; Feb;8(1):e000484

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ABOUT THE AUTHORS Cindy Flower MBBS, DM, FACP Dr. Cindy Flower is a graduate of The University of the West Indies (UWI) - medical school in 1988 and postgraduate Internal Medicine program in 1993. She completed Fellowship training in Rheumatology at the University of Minnesota in 1995 and holds a Specialty Certificate in Rheumatology (UK).

She established the Rheumatology clinic at the Queen Elizabeth Hospital, Barbados, in 1997, and is currently an Associate Consultant in Rheumatology there. Dr. Flower is a Senior Associate Lecturer, Faculty of Medical Sciences, The UWI, Cave Hill Campus, Barbados. She teaches both medical students and Residents in Rheumatology.

In 2007, along with collaborators, she established the Barbados National Lupus Registry which documented the epidemiology and clinical features of lupus in the Barbadian population.

Her professional recognitions include, an international fellow of the American College of Rheumatology, a fellow of the American College of Physicians and a member of the Caribbean Association for Rheumatology.

Dr. Flower has been a member of the Medical Advisory Committee of the Hope Foundation of Barbados since 1995.

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Shelley Weir MBE, MSc. President of the Hope Foundation Shelley Weir established the Hope Foundation in 1991 to support patients with lupus and to educate the public and medical professionals about this disease with which she was diagnosed in 1990. In 1992, the Hope Foundation became the first patient-led organization to host an international medical conference and the tradition of education continues — from modest venues in small churches to school halls and conference theatres.

Ms. Weir’s unwavering talent and energy for advocacy has made her a much sought-after advisor supporting regional groups with her template for development of similar organizations. She has the distinction of being the first non-medical person to chair the Patient Care Committee of the Board of the Queen Elizabeth Hospital. Through her determination the Hope Foundation continues to grow and thrive.

The projects she has developed include seminars for newly diagnosed patients, annual walk for lupus as well as annual voices for lupus concert. Almost from inception she expanded the mandate of the Hope Foundation to include patients with sickle cell disease, arthritis and the lesser-known chronic diseases.

More recent projects developed by Ms. Weir include the Sherene Hillock-Hinds Caring for the Carer award celebrating caretakers, and the Lupus Essential Kit program to engage patients in self-management.

Ms. Weir holds a Master of Science degree in Non-Governmental Organization management from the London School of Economics and Political Science. In 2009, she was recognised by the Government of Barbados for her service as a community activist with a national honour — Member of the Order of the British Empire (MBE).

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MEDICINE

ISBN: 978-976-96783-1-6