BackgroundPaediatric 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.MethodsThis 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.ResultsFifty 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.ConclusionThe 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.

The development of ocular disorders associated with RA is associated with a significant negative impact on the quality of life of the patients.

Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disorder of unknown cause. It accounts for about one in five cases of SLE. The tendency for SLE to run a fulminant course when it starts in childhood has made JSLE a potentially more severe disease than adult SLE. Reports of JSLE from sub-Saharan Africa are scanty in spite of the increasing reports of adult SLE. We conducted a 4-year retrospective study of JSLE cases seen at the Lagos State University Teaching Hospital between January 2010 and December 2014. Out of the 12 patients studied, eight were girls and four were boys. All patients had positive antinuclear antibody and extractable nuclear antibody tests. Anti-dsDNA antibody was positive in 10 patients. Eight patients had renal disease while four patients had neuropsychiatric manifestations. Haematological abnormalities and constitutional symptoms were present in all patients. Patients were treated with pulse methylprednisolone, oral prednisolone, hydroxychloroquine and azathioprine. Three patients also received rituximab. In conclusion, JSLE exists in Nigeria and exhibits clinical and immunological characteristics similar to its pattern in other parts of the world. It is, however, diagnosed late and is possibly being underdiagnosed as there is no paediatric rheumatologist in the country.

Background The word scleroderma means ‘hard skin’ that develops due to excessive accumulation of collagen. It is the third most frequent rheumatic disease in paediatric rheumatology after juvenile idiopathic arthritis and systemic lupus erythematosus. When it occurs in individual <16 years, it is called juvenile scleroderma. It is a rare disease that occurs in one per million children, documented to be more common in African adults with poorer survival states compared with Caucasians. Objectives To describe the clinical and laboratory characteristics of children with juvenile scleroderma seen in our clinic, thus, increasing its awareness. Methods Retrospective review of records of three children diagnosed with juvenile scleroderma at the paediatric Rheumatology Clinic of Lagos State University Teaching Hospital(LASUTH) between May 2018 to April 2022. Results Case 1 A 12-year-old girl presented with skin tightness of the left hand and thigh of one year, contracture of the 3rd, 4th and 5th proximal interphalangeal joints, arthritis of the left wrist, skin induration of the dorsum of the left hand involving the 4th and 5th finger and extending to the wrist and skin and induration of the anterolateral aspect of the left thigh. Her blood tests showed an erythrocyte sedimentation rate (ESR) at 20 mm/h, an ANA titre of 1:2560, a negative anti-Scl 70/anti-centromere antibodies, a normal complete blood count and serum electrolytes/urea/creatinine. A diagnosis of linear scleroderma was made, the patient had prednisolone, methotrexate and folic acid, in addition to topical emollients. She improved clinically as observed during follow up visits six weeks after initiation of treatment but later defaulted from the clinic due to unknown reason. Case 2 A 4-year-old girl presented with constitutional symptoms, swollen hands and feet, sclerodactyly, narrowing of oral aperture, ulcers at tips of the fingers, inflammatory pain of the large joints, hypopigmented macules on the face, trunk, abdomen and back and also abnormal capillaroscopy. Erythrocyte sedimentation rate was 22 mm/h with normal levels of electrolytes/urea/creatinine, thrombocytosis, ANA titre of 1:640 and negative anti-centromere and anti-U1RNP antibodies. A diagnosis of diffuse systemic sclerosis was made. She started prednisolone, methotrexate, nifedipine and omeprazole and was asked to do an ECG, an Echocardiogram, a spirometry and a chest HRCT but she couldn’t afford to do these investigations due to severe financial constraints. She was clinically stable for four months until she presented at the emergency room with sudden loss of consciousness and congestive cardiac failure. She died during resuscitation attempt. Case 3 An 11-year-old girl known patient of haematology unit with sickle cell anaemia, presented with inflammatory arthritis of the small joints of the hands, elbows and knees of nine-month duration, sclerodactyly, contractures of the PIP of the fingers, narrowing of oral aperture, generalized hypopigmented macules and abnormal nailfold capillaroscopy. Investigation showed an ANA titre of 1:640, positive anti-Scl 70 antibodies, negative anti-centromere antibodies, ESR of 130 mm/h and thrombocytosis. The echocardiogram showed a normally structured heart with severe restriction on spirometry and features of interstitial lung disease on HRCT of the chest. She started mycophenolate mofetil, prednisolone and nifedipine. She later received 2 doses of rituximab due to slow clinical improvement. She is being followed up. Conclusion Most data on scleroderma are from adult studies, we reported these three cases due to the rare occurrence of scleroderma in children, thus increasing its awareness.

Background Juvenile Systemic lupus erythematosus (JSLE) is a chronic multisystem autoimmune disease of childhood, which accounts for 10% to20% of systemic lupus erythematosus (SLE). It was initially thought that systemic lupus erythematosus (SLE), including JSLE, was rare in Blacks, this was eventually debunked with increasing reports from Africa. However, it is now known that SLE is more common among patients of African descent in western countries. While the estimated prevalence of JSLE in the developed countries is 0.36–2.5 per 100 000, data in Black Africans is scarce due to missed diagnosis, poor diagnostic capacity and under-reporting. JSLE has protean manifestations similar to common paediatric conditions such as severe malaria, overwhelming septicaemia, hyper-haemolytic crisis in sickle cell anaemia etc., which often cause delayed or missed diagnosis. The objective is to describe the demographic and clinical characteristics, including outcome of children with JSLE, thus raising awareness on their occurrence and management in Nigerian children. Methods Retrospective review of records of children diagnosed with SLE at the Adult/paediatric Rheumatology Clinic and Paediatric Wards of Lagos State University Teaching Hospital (LASUTH) from May 2018 to May 2021. Results Twenty-two children, nineteen (n = 19) girls and three (n = 3) boys, aged 5–17 years, fulfilled the American College of Rheumatology (ACR)’s diagnostic criteria for JSLE out of 45 children newly diagnosed with paediatric rheumatic diseases during this period. The duration of symptoms before diagnosis ranged from two weeks to three years. The presentations included recurrent severe anaemia (n = 16), arthritis (n = 17), arthralgia (n = 17), malar rash (n = 17), neurologic symptoms (n = 5) oral ulcers (n = 17), cardiopulmonary symptoms (n = 5), photosensitivity (n = 10) and renal disease (n = 14). Laboratory findings included elevated ESR with a mean (±SD) of 99.68 ± 44.44, positive ANA (n = 22), positive anti-dsDNA (n = 12), low C3 & C4 (n = 2), positive anti-Smith antibody (n = 8) and massive proteinuria (n = 14). All patients were treated with steroids and disease modifying anti-rheumatic drugs (synthetics and biologics) based on disease severity and organ manifestations. Sepsis (n = 4) was the most common preliminary diagnosis before a final diagnosis of JSLE was made, other preliminary diagnosis were pulmonary tuberculosis (n = 1), dermatitis (n = 1), acute glomerulonephritis (n = 1), Typhoid fever (n = 1), malaria (n = 1), deep vein thrombosis (n = 1), seizure disorder (n = 1), leukaemia (n = 1), meningitis (n = 1), meningoencephalitis (n = 1), hyper-haemolytic crisis in sickle cell anaemia (n = 1), Steven Johnson syndrome (n = 1), Juvenile Idiopathic Arthritis (n = 2), Eczema (n = 1), unexplained anaemia (n = 1) and acute rheumatic fever (n = 1). One boy and three girls defaulted from clinic after commencement of treatment due to severe financial constraints of their parents and religious beliefs, however six girls died, four from an acute flare and two from end stage renal disease. Conclusion Our study has shown that JSLE has protean manifestations with a tendency to miss its diagnosis due to similarity of signs and symptoms with common childhood diseases in our environment. JSLE may not be as rare as commonly thought, thus its prompt diagnosis and treatment require a high index of clinical suspicion.

BackgroundKeratoconjunctivitis sicca (KSC) is the most frequent ocular manifestation of rheumatoid arthritis (RA). It is highly heterogenous and exhibits a considerable variability of presentation. Whereas a significant proportion of patients with KCS may be asymptomatic; it is unknown if the degree of dryness of the eyes, irrespective of symptomatology, has an association with the overall quality of life and functional ability.ObjectivesTo determine the correlation between the Schirmer’s score and the Medical Outcome Study 36-Item Short Form Health Survey (SF-36) score as well as the disability index measured by the Health Assessment Questionnaire-Disability Index (HAQ-DI).MethodsA total of 50 Nigerian patients satisfying the 2010 American College of Rheumatology/European League Against Rheumatism classification criteria for RA were studied. Tear production was measured by unstimulated 5 min Schirmer’s test (using 5 mm by 35 mm Whatman filter paper) and ocular staining with flourescein stain. Each patient also completed the SF-36 and HAQ-DI questionnaires.ResultsThe mean age of the patients was 47.2±12.5 years. Among them, 42 (84%) were females, giving a female-to-male ratio of 5.25:1. Rheumatoid factor and anti-citrullinated protein antibody were positive in 38 (76%) and 30 (60%) patients respectively. KCS was found in 15 (30%) patients among whom only 6 patients were symptomatic for dry eyes. The mean visual acuity [LogMAR (Snellen equivalent)] among the patients with and without KCS were 0.70 (6/30) and 0.10 (6/7.5). Among all patients, there was a positive correlation between the mean Schirmer’s score (between each patients two eyes) and the Physical Component Summary (r=0.292, p=0.008) as well as the Mental Component Summary (r=0.228, p=0.030) of the SF-36. There is however no significant correlation between the mean Schirmer’s score and the HAQ-DI (r=0.148, p=0.188).ConclusionsOcular manifestation is very common in RA and KCS is particularly rampant. Even in asymptomatic patients, the degree of xerophthalmia may give a reliable insight into both the physical and mental quality of life but not into the degree of functional disability.References[1] Vignesh APP, Srinivasan R. Ocular manifestations of rheumatoid arthritis and their correlation with anti-cyclic citrullinated peptide antibodies. Clin Ophthalmol2015;9:393–7.[2] Bron AJ, Tomlinson A, Foulks GN, et al. Rethinking dry eye disease: a perspective on clinical implications. Ocul Surf2014;12(2):S1-S31.Disclosure of InterestNone declared

ObjectivesTo highlight common precipitants and co-morbidities of gout in Nigerians; determine the frequency of chronic kidney disease (CKD) in Nigerian gout patients, as well as identify significant associations of CKD in gout patients.Material and methodsRetrospective cross-sectional study of gout cases seen at the Rheumatology Clinic of the Lagos State University Teaching Hospital over five years from January 2011 to December 2015. Gout was diagnosed using the 1977 American Rheumatism Association (ARA) criteria. Clinical and laboratory data were extracted and examined for the presence of CKD defined using Kidney Disease Improving Global Outcomes (KDIGO) 2012 guidelines as estimated glomerular filtration rate (eGFR, CKD-EPI) < 60 mls/min/1.73 m2 body surface area for > 3 months.ResultsOne hundred and six gout patients were identified representing 4.5% out of a total of 2330 cases seen during the study period. There were 94 males and 12 females. Oligoarthritis was most frequent (41.5%) with the knee mostly affected (20.1%). Diuretic use was the most frequent precipitant (37.9%) with hypertension the commonest co-morbidity (62.9%). Of 70 patients with complete results, 29 had eGFR (CKD-EPI) < 60 mls/min (41.4%). Age, proteinuria, body mass index (BMI) and packed cell volume (PCV) were significant associations of CKD. Gout patients with CKD were significantly older (61.79 vs. 54.41 years, p = 0.003) with a significantly higher proportion developing proteinuria (15 vs. 4 patients, p ≤ 0.0001) compared to those without CKD. In contrast, those with CKD had a significantly lower BMI (27.31 vs. 29.65 kg/m2, p = 0.026) and PCV (31.97 vs. 37.95%, p = 0.005).ConclusionsThiazide diuretic use is the most common precipitant while hypertension is the commonest comorbidity in Nigerian gout patients. About two in five Nigerian gout patients had chronic kidney disease at presentation with age, proteinuria, BMI and PCV as significant associations. It is thus imperative to screen for chronic kidney disease when managing gout patients.

Psoriatic arthritis is rare among Nigerians and predominantly affects males in their fourth decade. Oligoarthritis is common, and extra-articular manifestations are frequent.