Camptodactyly-Arthropathy-Coxa Vara-Pericarditis Syndrome: Important Differential for Juvenile Idiopathic Arthritis: A Report of Ten Cases

الملخص

The main objectives of this study is to describe the clinical features in patients with campatodactyly-arthropathypericarditis-coxa vara syndrome (CACP); highlight the important clinical and radiological features that allow
differentiation of CAPA from juvenile idiopathic arthritis; and to recognize the importance of differentiating
CAPA from juvenile idiopathic arthritis.
Ten affected children with CAPA from five different families following in pediatric rheumatology clinic, Tripoli
children hospital underwent complete history, complete clinical examinations, laboratory and radiological
investigations and histological study of synovium in one case.
All cases were Libyans, age 6 to 19 years, 3 cases were male and 7 cases were female. Campatodactyly developed
at birth in 3 cases, 5 cases developed campatodactyly before the age of 5 years and 2 cases do not have it. Coax
vara was present in 5 cases, carditis was detected in 3 cases with varying severity, and arthropathy was present in
all cases with multiple joint involvements. Consanguinity was positive in 8 cases, 2 cases have no consanguinity
but they came from the same village. Family history of same disease was positive in all cases. All the cases were
diagnosed wrongly as acute rheumatic fever or juvenile idiopathic arthritis and treated accordingly. Laboratory
investigations (CBC, ESR, CRP, ANA, RF) were normal in all cases.
Plain hip radiology revealed short broad femoral neck and widening of joint space in all cases; no evident cartilage
destruction excited in any case, MRI of the hips was done in two cases, showed rim-like enhancement of the lining
of the fluid filled bursa in both of them. Histopathology revealed non inflammatory synovial hyperplasia with
multinucleated giant cell in the only case in which synovial biopsy was performed.
Our cases represent a familial autosomal recessive syndrome of non-inflammatory arthropathies associated with
campatodactyly and coax vara, the complete picture of the disease may be related to the disease duration. Plain X
rays, MRI and synovial biopsy are useful tools in the diagnosis.
Physicians should be aware of the symptoms to avoid mis diagnosis with pediatric rheumatology conditions
especially juvenile idiopathic arthritis, and CACP should be considered in all patients who present with noninflammatory arthropathy particularly if radiograph reveal absence of erosions.