The authors have declared that no competing interests exist.
To describe the frequency, clinico-laboratory characteristics and treatment outcomes of patients with juvenile idiopathic arthritis (JIA) in Lagos State University Teaching Hospital (LASUTH), Lagos, Nigeria.
This is a retrospective review of patients with JIA seen over a five-year period at the rheumatology clinic and children ward of LASUTH. We reviewed the folders of 28 patients from our unit records. The demographics, baseline clinical and laboratory characteristics, treatment given and patient outcomes were extracted and analyzed.
A total of 28 patients with JIA were managed over the study period. Twenty one (75%) patients among our JIA cases were female and the mean age at presentation was 9.8±3.9 years. The mean duration of symptoms before diagnosis was 21.8±5.7 months. Polyarticular JIA (PJIA) constituted 14 (50%) cases, while oligoarticular and systemic-onset JIA (SoJIA) constituted 9 (39.3%) and 5 (17.9%) of the JIA cases respectively. Anaemia was present in 20 (71.4%) patients, leucocytosis in 16 (57.1%) and thrombocytosis in 11 (39.2%). Twenty five (89.2%) patients had elevated erythrocyte sedimentation rates (ESRs), 21 (75%) had elevated C-reactive protein levels and 23 (82.1%) patients had hyperferritinaemia. Positive antinuclear antibody (ANA) was found in 5 (17.8%) patients. Mortality was documented in 2 (7.1%) patients both of whom were SoJIA cases. Eleven (39.3%) patients were lost to follow up.
Unlike the common report of oligoarticular JIA (OJIA) being the most frequent subtype of JIA in various series from North America and Europe, PJIA was the most frequent subtype seen among our patients and this variant accounted for half of all JIA cases seen. There were no cases of psoriatic, enthesitis-related or undifferentiated JIA and most patients had haematological abnormalities and high levels of inflammatory markers at presentation.
Juvenile idiopathic arthritis (JIA) is the most common cause of chronic arthritis in children and it is a cause of considerable morbidity and disability. According to the International League of Associations for Rheumatology (ILAR) definition of JIA, any arthritis lasting at least 6 weeks and developing before the 16th birthday in the absence of a known cause is classified as JIA (1). While the burden and impact of JIA are known across the developed world (2), the topic is still evolving in Africa where more emphasis is on communicable than non-communicable diseases. This double burden of diseases is mounting in Africa and it has been projected that non-communicable diseases will cause more than 60% of all mortalities by the year 2030 (3). A systematic review of studies outside Africa reported a prevalence of JIA ranging from 0.000038% to 0.004% (4). The prevalence among European and North American children populations ranges from 16 to 150 per 100,000 (5). The few reports of JIA in sub-Saharan Africa have been hospital based
This is a retrospective review of case records of JIA managed from June 2010 to April 2016 at the adult rheumatology unit of Lagos State University Teaching Hospital (LASUTH). LASUTH is one of the two tertiary teaching hospitals in the cosmopolitan city of Lagos and it has one of the two rheumatology clinics in the city. There is no pediatric rheumatologist in Nigeria and as such JIA and other paediatric rheumatic diseases are managed by adult rheumatologists in conjunction with paediatricians. The paediatric patients with either musculoskeletal complaints or systemic symptoms suspicious of a rheumatic disease were referred for rheumatology review from the paediatric clinic, peripheral hospitals or paediatric wards. The diagnosis of JIA and its sub-groups were based on the ILAR classification criteria. The entry criterion for case selection was the diagnosis of JIA in the patient’s record before 16 years of age. The hospital records were retrieved for each patient and details of biodata, baseline clinical parameters, baseline laboratory indices, drug treatments and patient outcomes were extracted. The duration of illness was taken as the onset from the first symptoms to clinical diagnosis at our facility. The full blood count, haematocrit, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), serum ferritin, and antinuclear antibody (ANA) were recorded in all patients. Rheumatoid factor (RF) was tested in 7 patients who had the diagnosis of polyarticular JIA (PJIA). ANA and RF were analyzed by Enzyme-linked immunosorbent assay (ELISA) and nephelometry methods respectively.
Descriptive statistics was used to analyze categorical and continuous variables. The categorical variables were presented as frequencies and percentages while continuous variables were presented as mean and standard deviation. Ethical approval was granted by the research and ethics committee of LASUTH.
A total of 28 patients with JIA, which included 25 cases referred from the general paediatric clinic, 2 cases diagnosed in paediatric ward and 1 case referred from a peripheral hospital were managed at the rheumatology unit. These accounted for 1.2% of the 2330 rheumatic disease cases managed over the study period. As shown in
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Female | 21 (75) | ||
Male | 7 (25) | ||
Age at presentation (years) | 1.5-15 | 9.8 (±3.9) | |
Age at onset (years) | 1.3-14.3 | 8.1 (±4.0) | |
Duration of illness (months) | Mar-60 | 21.8 (±5.7) | |
Constitutional symptoms | 15 (53.6) | ||
Only small-joint involvement (number of patients) | 3 (10.8) | ||
Large and small-joint involvement (number of patients) | 9 (32.1) | ||
Only large-joint involvement (number of patients) | 16 (57.1) | ||
Extra-articular features | 7 (25) | ||
Polyarticular JIA | 14 (50) | ||
Oligoarticular JIA | 9 (32.1) | ||
Systemic-onset JIA | 5 (17.9) | ||
Lost to follow up | 11 (39.3) | ||
Mortality | 2 (7.14) |
JIA=Juvenile idiopathic arthritis
As shown in
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9.9±3.4 | 8.3±2.8 | 12±2.4 |
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7.9±3.6 | 6.9±4.9 | 10.2±3.2 |
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22.9±16.7 | 12.5±13.9 | 21.6±10.0 |
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12/2 | 7/2 | 2/3 |
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7(50) | 3(33.3) | 5(100) |
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0(0) | 0(0) | 3(60) |
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6(42.9) | 4(44.4) | 3(60) |
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1(7.1) | 1(11.1) | 0(0) |
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2(14.3) | 1(11.1) | 1(20) |
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3(21.4) | 2(28.6) | 0(0) |
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10(71.4) | 5(55.6) | 5(100) |
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9(64.3) | 3(33.3) | 4(80) |
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6(42.9) | 2(28.6) | 3(60) |
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12(85.7) | 8(88.9) | 5(100) |
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11(78.6) | 5(55.6) | 5(100) |
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1(7.1) | 3(33.3) | 1(20) |
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10(71.4) | 8(88.9) | 5(100) |
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2(14.3) | 0(0) | 0(0) |
JIA=juvenile idiopathic arthritis, SD=standard deviation, ESR=erythrocyte sedimentation rate, CRP=C-reactive protein, ANA=anti-nuclear antibody, NSAID=Non-steroidal anti-inflammatory drug.
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Intra-articularsteroid treatment n (%) | 3(21.4) | 6(66.7) | 1(20) |
NSAID treatment n (%) | 14(100) | 9(100) | 5(100) |
Methotrexate treatmentn (%) | 14(100) | 9(100) | 5(100) |
Biologic treatment n (%) | 1(7.1) | 0(0) | 0(0) |
Lost to follow up n (%) | 5(35.7) | 4(44.4) | 2(40) |
Mortality n (%) | 0(0) | 0(0)7 | 2(40) |
JIA=juvenile idiopathic arthritis, NSAID=Non-steroidal anti-inflammatory drug.
All patients were treated with non-steroidal anti-inflammatory drugs (NSAIDs) and methotrexate. Many of these patients had been on self-prescribed NSAIDs before diagnosis. Etanercept was administered in one refractory RF-positive PJIA case. There were 2 mortalities accounting for 7.14% of the JIA cases while 11 patients were lost to follow up. Both deaths were in patients with SoJIA. While one patient died from overwhelming sepsis, the other died from suspected macrophage activating syndrome (MAS).
We studied 28 cases of JIA which constituted 1.2% of the 2330 rheumatic cases diagnosed and managed in a public rheumatology unit over a 6-year period. The scarcity of paediatric rheumatologists in Africa is a challenge for the early detection and appropriate management of childhood-onset rheumatic diseases. There is currently no paediatric rheumatologist in Nigeria and suspected and confirmed cases of paediatric rheumatic diseases are managed by other paediatricians and adult-patient rheumatologists. Accordingly, the awareness and indices of suspicion for paediatric rheumatic diseases are very low among Nigerian clinicians. It is unknown, how large the annual number of missed paediatric rheumatic diseases is and what burden this might be contributing to the morbidity and mortality due to childhood non-communicable diseases.
The high mean age of onset of JIA observed in this study is similar to findings recorded in other African studies
The female-male ratio of 3:1 found in our study is larger than the range of 1-2.3:1 found in most other studies
A high proportion of our patients, irrespective of subtype, had prominent constitutional symptoms and elevated inflammatory markers. This finding is in agreement with the observation of Oyoo et al
Many of our patients had been on NSAIDs and in a few cases oral prednisolone before presentation. Suitable adjustments were made in these drugs and all patients were commenced on subcutaneous or oral methotrexate according to our local policy. Intra-articular steroids were administered in few cases. The only JIA-suitable biologic available in Nigeria is etanercept and only one in six patients eligible for it were able to afford it. Affordability of biologics and other emerging treatment modalities for JIA is a huge barrier to the optimal treatment of Nigerian patients. The only other biologic currently available in Nigeria, rituximab, has not been approved for JIA but anecdotal reports have documented its efficacy in refractory SoJIA
Mortality in JIA has been shown to be higher than in the general population, and especially, higher among individuals with SoJIA than other subtypes
JIA may not be so rare in Nigeria and the complete absence of paediatric rheumatologists in the country may have contributed to the low awareness and detection of these diseases among clinicians. Similar to many other reports from across Africa, PJIA constitutes the largest proportion of JIA among Nigerian patients. The care of these patients is plagued with several challenges including the often late presentation, high disease activities, lack of adequate healthcare financing and resources and the scarcity of specialists. Prospective studies on the subject will be helpful to determine the true burden and course of JIA among Nigerians.