African League Against Rheumatism (AFLAR) preliminary recommendations on the management of rheumatic diseases during the COVID-19 pandemic.

OBJECTIVES: To develop recommendations for the management of rheumatic and musculoskeletal diseases (RMDs) during the COVID-19 pandemic. METHOD: A task force comprising of 25 rheumatologists from the 5 regions of the continent was formed and operated through a hub-and-spoke model with a central working committee (CWC) and 4 subgroups. The subgroups championed separate scopes of the clinical questions and formulated preliminary statements of recommendations which were processed centrally in the CWC. The CWC and each subgroup met by several virtual meetings, and two rounds of voting were conducted on the drafted statements of recommendations. Votes were online-delivered and recommendations were pruned down according to predefined criteria. Each statement was rated between 1 and 9 with 1-3, 4-6 and 7-9 representing disagreement, uncertainty and agreement, respectively. The levels of agreement on the statements were stratified as low, moderate or high according to the spread of votes. A statement was retired if it had a mean vote below 7 or a 'low' level of agreement. RESULTS: A total of 126 initial statements of recommendations were drafted, and these were reduced to 22 after the two rounds of voting. CONCLUSIONS: The preliminary statements of recommendations will serve to guide the clinical practice of rheumatology across Africa amidst the changing practices and uncertainties in the current era of COVID-19. It is recognized that further updates to the recommendations will be needed as more evidence emerges. Key Points • AFLAR has developed preliminary recommendations for the management of RMDs in the face of the COVID-19 pandemic. • COVID-19 is an unprecedented experience which has brought new concerns regarding the use of some disease-modifying anti-rheumatic drugs (DMARDs), and these recommendations seek to provide guidelines to the African rheumatologists. • Hydroxychloroquine shortage has become rampart across Africa as the drug is being used as prophylaxis against COVID-19 and this may necessitate a review of treatment plan for some patients with RMDs. • Breastfeeding should continue for as long as possible if a woman is positive for SARS-CoV-2 as there is currently no evidence that the infection can be transmitted through breast milk.

Tossing and turning with degenerative arthropathy: an assessment of poor sleep quality in knee osteoarthritis.

OBJECTIVES: To determine the frequency and predictors of sleep abnormalities among patients with knee osteoarthritis (OA) in Nigeria. MATERIAL AND METHODS: A multi-centre, hospital-based, cross-sectional study, involving 250 knee OA patients. Consenting patients 18 years and above, who satisfied the American College of Rheumatology (ACR) criteria for knee OA were recruited from five Nigerian tertiary centres over 3 months. An interviewer-administered questionnaire was used to collect demographic and relevant clinical information. The Pittsburgh Sleep Quality Index (PSQI) was used to assess sleep quality with scores ≥ 5 indicating poor sleep. Other variables assessed were pain, depression, functional class and family functioning. Data were summarized using appropriate measures of central tendency and dispersion. Multiple logistic regression analysis was done to identify predictors of poor sleep. Analysis was done using SPSS version 21.0 with p < 0.05 considered significant. Study approval was obtained from the ethical committees of each of the study sites. RESULTS: Participants included 209 females (83.6%) with mean age 59.9 ±10.6 years. One hundred and forty-one participants (56.4%) had PSQI scores ≥ 5 (poor sleep). This was significantly associated with depression (p < 0.001), level of education (p = 0.001), higher pain scores (p < 0.001), body mass index (p = 0.040), medial knee OA (p = 0.032) and patello-femoral OA (p = 0.002). Higher level of education, worse depression scores and higher WOMAC pain scores were the best predictors of poor sleep quality. CONCLUSION: Sleep quality was poor in over half of our knee OA patients and best predicted by depression, pain and level of education. Regular sleep quality assessment for knee OA patients is recommended.

Rowell Syndrome in Nigeria: Systemic Lupus Erythematosus Presenting as Recurrent Erythema Multiforme in a Young Woman.

Dear Editor, Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by diverse patterns of auto-antibody production with multi-organ affectation. Cutaneous involvement, either alone or in association with other systemic illnesses, is one of its most common manifestations (1). Dermatologic disorders like malar and discoid rashes are quite suggestive of SLE. However, the occurrence of non-specific skin lesions like erythema multiforme (EM) in patients with SLE (Rowell syndrome) can rarely occur (1). In such patients, a diagnosis of SLE may be missed or delayed in the absence of other overt clinical features of lupus. Herein we report a case of recurring EM-like eruptions as the cardinal cutaneous manifestation of previously undiagnosed, active SLE in a young Nigerian woman. A 26-year-old Nigerian woman presented with a three-day history of non-pruritic, generalized, and target-like, erythematous annular patches and plaques which mostly affected the trunk. A few lesions had presented with crusting and erosions at the time of examination (Figure 1). Associated symptoms included oral painful ulcers, low grade fever, and malaise. The patient had no other systemic symptoms and her prior drug history was not remarkable. Her erythrocyte sedimentation rate (ESR) was 66 mm/hour using the Westergren method. Screening for HIV and hepatitis B and C was negative. Herpes simplex, cytomegalovirus, and Epstein Barr viruses could not be screened for. Other baseline investigations (complete blood count, electrolytes, urea and creatinine as well as urinalysis) were within normal limits. The patient was managed as a case of EM of an unidentified inciting agent and her symptoms resolved with supportive care and antibiotics. However, she developed a recurrence about 5 weeks later, with more extensive and coalescent skin lesions (Figure 2). Additionally, there was a new onset of alopecia and pain in the small joints of the hands as well as both knees and ankles. At this time, the patient's ESR had gone up to 112 mm/h and she had developed significant proteinuria, with a protein creatinine ratio of 1.3 g/g (reference <0.5 g/g). Her antinuclear antibody (ANA) titer was high (1:320) with a speckled pattern. Anti-Smith antibody was also positive. A renal biopsy was declined. A tentative diagnosis of Rowell syndrome was made. The patient was started on high-dose steroids and hydroxychloroquine 200 mg twice daily. Subsequent care included the use of mycophenolate mofetil 1 g twice daily for 6 months. This was then changed to azathioprine at 50 mg twice daily. Follow-up after 6 months showed sustained clearance of skin lesions, resolution of fever and joint pains, as well as improvement in the renal profile, with a urine protein-creatinine ratio of 0.77 g/g. The presence of systemic lupus erythematosus, EM-like lesions, and a speckled pattern of antinuclear antibody in our patient fulfils the revised diagnostic criteria for RS put forward by Zeitouni et al. at the turn of the twenty-first century (2). Considering the rarity of EM-like lesions in SLE and the possibility of constitutional symptoms in EM, a diagnosis of RS may be readily overlooked in patients like the one described, whose major cutaneous manifestation of severe active SLE was EM-like lesions. In contrast to classic EM, where skin lesions are concentrated in the extremities, a predominant truncal distribution of EM-like lesions as found in our patient may favor a clinical consideration of RS (3). However, some authors have challenged the existence of Rowell syndrome as a distinct clinical laboratory entity. Arguments put forward in this regard include the fact that none of the immunological markers that have been described in RS are specific to any disorder. Additionally, the annular polycyclic dermatosis seen in sub-acute cutaneous lupus erythematosus (SCLE) can be difficult to clinically and histologically differentiate from EM (4,5). Patients with SLE also have a higher likelihood of developing adverse drug reactions (6). The inherent complexity of SLE may make for delayed and oftentimes difficult diagnosis, especially in a country where immunologic tests are expensive and rheumatologists are scarce. When patients do occasionally present with recurrences of skin lesions in the spectrum of EM, Steven-Johnson syndrome, and toxic epidermal necrolysis in the absence of a definite inciting agent, undiagnosed lupus may indeed be present in some of these individuals and should be considered in the differential diagnosis. In conclusion, while it is very rare, SLE may present first with recurrent episodes of EM-like rash. Despite the various possibilities which underlie their association, prompt identification and treatment of SLE in patients presenting with EM is important to prevent death or irreversible organ damage.

How much gloom is in groans? Depression and its determinants in Nigerian patients with knee osteoarthritis: a multi-center cross-sectional study.

OBJECTIVES: To determine the prevalence of depression and its determinants among Nigerian patients with knee osteoarthritis (OA). METHODS: Two hundred and fifty patients satisfying the American College of Rheumatology classification criteria for knee OA were recruited from five centers. Pain was assessed using Western Ontario and McMaster Universities Index (WOMAC) pain subscale, functional status was determined by Steinbrokers criteria, radiographic knee OA was graded using Kellgren-Lawrence criteria, depression was determined using the Patient Health Questionnaire (PHQ-9), sleep quality by the Pittsburgh Sleep Quality Index (PSQI), and family functioning by Family APGAR (Adaptation, Partnership, Growth, Affection, and Resolve). Factors associated with depression in KOA following bivariate analyses were adopted as independent variables in logistic regression analysis to determine the predictors of depression in KOA. The study was approved by the Ethical Review Committee of each of the study centers. RESULTS: The mean age of patients was 59.90 ± 10.62 years and 209 (83.6%) were females. Their mean PHQ-9 score was 4.68 ± 4.19 with 105 (42%) having depression (PHQ-9 ≥ 5). Eighty-four (80%) of patients with depression had poor sleep quality (PSQI≥ 5.0). Depression was significantly associated with poor sleep, WOMAC pain scores, medial compartment KOA, lateral compartment KOA, and patellofemoral OA. Poor sleep quality was the best predictor of depression (OR 4.555, CI (2.241-9.257), p < 0.001) followed by moderate to severe pain (OR 2.490, CI (1.119-5.542), p < 0.025). CONCLUSION: Depression is common among patients with knee OA, and depression can be predicted by poor sleep quality and moderate to severe pain.

The impact of ocular manifestations of rheumatoid arthritis on the health-related quality of life and the functional ability of black Africans.

Objectives To determine the patterns, predictors and overall impact of ocular manifestations of rheumatoid arthritis (RA) on the health-related quality of life and disability index. METHODS: A total of 50 Nigerian patients with RA were studied. Full ocular evaluation was done to determine the presence of each defined ocular manifestation of RA. All patients completed the Medical Outcome Study 36-Item Short Form Health Survey (SF-36) and the Health Assessment Questionnaire-Disability Index (HAQ-DI) questionnaires. RESULTS: The mean ± SD age of the patients was 47.2 ± 12.5 years. A total of 42 cases of ocular diseases were found in 23 (46%) patients. Keratoconjunctivitis sicca was found in 15 (30%) patients; cataract, 13 (26%); scleritis/episcleritis, 4 (8%); disk edema, 4 (8%); glaucoma, 3 (6%); ulcerative keratitis, 1 (2%); vitreitis, 1 (2%); and macula edema, 1 (2%) patient. There is an association of the presence of ocular manifestations with the physical component summary (PCS) [T = - 3.398, P = 0.001] and the mental component summary (MCS) [T = - 2.616, 0.012] of the SF-36 but not with the HAQ-DI (T = 1.685, 0.099). Following multiple regression analysis, the predictors of the presence of ocular manifestations were age greater than 45 years and positive anti-citrullinated protein antibody. Following linear regression analyses, Steinbrocker's functional class independently predicted the PCS while both Steinbrocker's functional class and female sex predicted the MCS. CONCLUSIONS: The development of ocular disorders associated with RA is associated with a significant negative impact on the quality of life of the patients.

Thrombotic thrombocytopenic purpura as the first presentation in systemic lupus erythematosus.

Thrombotic thrombocytopenic purpura (TTP) is an uncommon, life-threatening disease characterized by severe thrombocytopenia, microangiopathic haemolytic anaemia, neurologic abnormalities, renal insufficiency, and fever commonly associated with infections, malignancy, drugs, and autoimmune diseases. Coexistence of TTP with systemic lupus erythematosus (SLE) is extremely rare, and the emergence of the full manifestation of SLE starting with TTP is even rarer. Black people due to genetic risk factors are at increased risk of TTP. In the present article the authors describe the case of a Nigerian woman who developed classical features of TTP as the first manifestation of SLE. The patient's condition was stabilized after treatment with intravenous pulses of methylprednisolone and subsequently on oral prednisolone and hydroxychloroquine treatment.

Is rheumatoid arthritis an innocent bystander in female reproductive problems? A comparative study of fertility in Nigerian women with and without rheumatoid arthritis.

OBJECTIVE: Contrary to the old belief that rheumatoid arthritis (RA) is rare in sub-Saharan Africa, recent reports have increasingly recognized that the burden of the disease has probably been long under-appreciated in West Africa. Thus, fertility and other attributes of the reproductive lives among women with RA have not received research attention in Africa. We aimed to compare the fertility between married Nigerian women with and without RA. METHODS: A comparative study of 50 women with RA and 50 women without RA was conducted via the specialist rheumatology clinics at two teaching hospitals in Nigeria. The participants were aged 18 years or older. Patients with RA were recruited on the basis of the fulfillment of the 1987 American College of Rheumatology classification criteria for RA, whereas the control participants were age-matched to the patients. Using an interviewer-administered questionnaire, demographic and clinical information was collected from each participant. Clinical details at the time of diagnosis of RA were extracted from the hospital records of the patients. The patients were tested for rheumatoid factor and anti-cyclic citrullinated peptide antibody, and the disease activity was assessed using the Clinical Disease Activity Index. The functional status was determined using the Steinbrocker functional classification. Statistical analysis was performed using Statistical Package for Social Sciences (SPSS) version 20 (IBM Corp.; Armonk, NY, USA). The proportions of individuals with history of infertility, irregular menstruation, and menopausal states were compared between the two groups using χ2 and Fisher's exact tests, whereas the durations of infertility and parities were compared using Mann-Whitney U and independent t-tests, respectively. RESULTS: A positive history of infertility was found in 22 (44%) patients and 14 (28%) controls (p=0.096), while 17 (34%) patients and 23 (46%) controls were found to be postmenopausal (p=0.221). History of irregular menstruation was present in 15 (30%) patients and 4 (8%) controls (p=0.005). The median duration of infertility was 60 (range: 16-132) months among the patients and 36 (range: 12-72) months among the controls (p=0.036), while the mean parity was 2.85±1.8 among the patients and 3.77±2.2 among the controls (p=0.027). A significant association was found between infertility and functional class as well as methotrexate treatment. CONCLUSION: Infertility is not uncommon among patients with RA, and like many aspects of rheumatic diseases, it may have been under-recognized in the Nigerian patients.

Hyperacute muscle weakness in an unusual coexistence of antisignal recognition particle and anti-Mi-2 antibodies.

Idiopathic inflammatory myopathies are a heterogeneous group of systemic diseases characterised by variable phenotypes of chronic progressive muscle weakness. Myositis-specific antibodies (MSAs) include antibodies to cytoplasmic signal recognition particle (SRP) and various tRNA synthetases as well as the nuclear helicase protein Mi-2. These antibodies are typically found only in a fraction of true myositis cases and they tend to be mutually exclusive. Few cases of coexistence of two MSAs in the same patient have been reported and these cases all involve an antisynthetase antibody coexisting with either anti-SRP or anti-Mi-2 antibody. Peculiar clinical manifestations may be associated with different combinations of MSAs but the rarity of these cases makes their characterisation difficult. We report the first ever case of anti-SRP and anti-Mi-2 copositive polymyositis in a 19-year-old boy who presented with a week history of profound muscle weakness that attained its peak within 24 hours of onset.

Spectrum of paediatric rheumatic diseases in Nigeria.

BACKGROUND: Paediatric rheumatology service in Sub-Sahara African is virtually not available as there is a shortage of paediatric rheumatologists and other rheumatology health professionals. We aim to describe the clinical spectrum and the frequencies of paediatric rheumatic diseases (PRDs) in Lagos State University Teaching Hospital (LASUTH), Lagos, Nigeria. METHODS: This is a retrospective review of patients with PRDs seen over a five year period (March 2010 to February 2016) at the rheumatology clinic and children ward of LASUTH. We reviewed the folders of 57 patients from our records. The demographics, baseline laboratory features, clinical diagnosis, treatment patterns and patient outcomes were extracted and analyzed. Clinical and laboratory characteristics between patients with Juvenile idiopathic arthritis (JIA) and patients with juvenile connective tissue diseases (JCTD) were compared using Fisher's exact test. RESULTS: Fifty seven patients were studied with a female to male ratio of 3 to 1 (Female: 43; M: 14). The mean age at presentation in years was 14 ± 4.4 years (range: 1.5-22 years). The mean duration of symptoms before diagnosis was 18.4 ± .9 months (range: 2-60 months). The diagnostic types of PRDs included 28(49.1%) cases of JIA. These were made up of 14 cases of polyarticular JIA, nine cases of oligoarticular JIA and 5 cases of systemic onset JIA. Others were 18 (24.6%) cases of juvenile systemic lupus erythematosus (JSLE), 3 (5.3%) cases of joint hypermobility syndrome, 2 (3.5%) cases of juvenile systemic sclerosis, 2 (3.5%) cases of fibromyalgia, 2 (3.5%) cases of plantar fasciitis, 1 (1.6%) case of juvenile dermatomyositis (JDM), 1 (1.6%) case of juvenile polymyositis-systemic lupus erythematosus (PM-SLE) overlap, 1 (1.6%) case of secondary bilateral knee osteoarthritis from Blount disease, 1 (1.6%) case of secondary osteoporosis from childhood leukemia and 1 (1.6%) case of Osgood-Schlatter's disease. Constitutional symptoms and extra-articular diseases were significantly more frequent among JCTD cases than among the JIA cases (Constitutional symptoms: 100% vs 83.3%, p = 0.003; extra-articular disease: 100% vs 10.7%, p = 0.001). The percentage mortality in this study was 10.5% while 20 (35.1%) of the patients were lost to clinic follow up. CONCLUSION: The pattern of PRDs observed in this study is similar to that described in South African and North American series but it differs from patterns reported in Asian series. Although hitherto largely unrecognized, PRDs may constitute a substantial cause of morbidity and mortality in black Africans.

When nuisance is nice: ignored erythema nodosa heralding the Löfgren's syndrome in a Nigerian woman.

Löfgren's syndrome (LS) is a variant of sarcoidosis characterised by the triad of erythema nodosum (EN), radiographic bilateral hilar adenopathy, and arthralgia/arthritis. Like all cases of sarcoidosis, it is of unknown aetiology and may constitute a diagnostic difficulty in the ambiguous phenotype. Löfgren's syndrome is associated with a good prognosis and commonly undergoes spontaneous remission within four months. However, the co-existence of multiple good and adverse prognostic factors in a patient may call for guarded expectation. Sarcoidosis is generally more prevalent among people of African descent, but the vast majority of the literature on sarcoidosis are from the western hemisphere. Löfgren's syndrome has been rarely documented in West Africans despite the availability of some reports of sarcoidosis in the region. We present a case of a Nigerian woman with LS that started out as isolated EN, which was ignored for months until the onset of florid pulmonary and systemic symptoms.

Recurrent spontaneous subcutaneous emphysema in a patient with rheumatoid arthritis.

Pulmonary air leak syndromes are extremely rare complications of systemic autoimmune connective tissue diseases and the occurrence of spontaneous subcutaneous emphysema (SSE) from pulmonary leak in the absence of pneumothorax or pneumomediastinum is even rarer. We report a case of recurrent SSE in a patient with rheumatoid arthritis and interstitial lung disease. The SSE was managed conservatively each time and it resorbed over several days. There has been no previous documented report of SSE in the absence of pneumomediastinum, pneumothorax or pulmonary nodules in a patient with RA.

Adult-onset Still's disease in a Nigerian woman.

Adult-onset Still's disease (AOSD) is an uncommon systemic inflammatory disorder of unknown aetiology. Although there have been reports and series elsewhere, there have been very few such reports on black Africans. The rarity of the reporting of this disease has been associated with a low index of suspicion and hence delayed diagnosis in patients suffering from it. We report a case of a 28-year-old woman, a teacher, who had been repeatedly treated for malarial fever over a 2-month period. She was also briefly managed elsewhere for systemic lupus erythematosus due to a persistent fever with associated polyarthralgia, sore throat, rash and high erythrocyte sedimentation rate. On presentation to our facility, she fulfilled the Yamaguchi criteria for AOSD and had a markedly elevated serum ferritin level. She was successfully managed with etanercept and methotrexate. This is the first report of AOSD from Nigeria.