Juvenile rheumatoid arthritis: the Saudi experience

We retrospectively studied the charts of 115 Arab children with juvenile rheumatoid arthritis [JRA], all of whom satisfied the American College of Rheumatology [ACR] criteria for JRA. They were followed between 1978 and 1993 at King Faisal Specialist Hospital and Research Centre and King Khalid University Hospital in Riyadh, Saudi Arabia. All patients were followed up for at least 18 months. The female to male ratio was 1.2:1, and the mean age of onset of the disease was six years. Ninety of the patients were Saudis and the remainder were Middle Eastern Arabs. The mode of onset was systemic in 44%, polyarticular in 30%, and periarticular in 26%. Chronic uveitis was found in only two of the children [1.7%]. Antinuclear antibody [ANA] was determined in 96 patients and was positive in 29 [30%]. Amyloidosis was not detected in this study population. The clinical and laboratory manifestations of this disease are presented. This review shows that the spectrum of clinical presentation differs in Arab children from those in the West. Systemic and polyarticular onset subtypes were more common, and the incidence of uveitis and amyloidosis was lower. Whether this reflects a genuine difference in the pattern of the disease, or is due to bias in referral pattern, remains to be detected. The answer is currently being sought through an ongoing prospective study

Juvenile systemic lupus erythematosus in 60 Saudi children

A ten-year retrospective analysis of the clinical features and survival of 60 Saudi children with systemic lupus erythematosus [SEE] was made. All the patients fulfilled the 1982 American College of Rheumatology's revised criteria for SLE and had had the disease at or before the age of 16 years. The female to male ratio was 5:1, the mean age of onset was 12.1 years [range 1.6-16 years], and the mean duration of follow-up was 4.7 years [range 2.2-11]. Thirty-eight patients [63%] were diagnosed correctly before referral to KFSH and RC or KKUH. The mode of presentation was as follows: 55 patients had musculoskeletal involvement [91.6%], 49 patients had skin involvement [81.6%], 40 patients had hematological abnormalities [66.6%], 39 patients had renal disease [65%], 10 patients had pulmonary involvement [16%], 23 patients had cardiovascular disease [38%] and 18 patients had central nervous system involvement. During the study period four patients died [6.6%] two of renal failure, one from meningitis and one from severe sepsis. This is the largest collection of childhood systemic lupus erythematosus from the Middle East and it shows that SEE is more common in Saudis than was hitherto believed, and that it has a high rate of organ involvement

Syndrome of camptodactyly, arthropathy and coxa vara [CAC syndrome]

Three Saudi children [two female, one male] are described who presented with familial arthropathy associated with congenital camptodactyly. This rare but recognized clinical entity has a variable clinical presentation and may be associated with pericarditis and coxa vara. Camptodactyly was observed in the neonatal period in all patients, while joint swelling was observed between the third and 11th month. Pericarditis was suspected in the referral hospital in one patient but was not subsequently confirmed at our institution, raising the possibility that pericarditis may be reversible. Radiological examination of the hips showed coxa vara with short femoral neck in all patients. Synovial biopsy in the three patients revealed proliferating synovial epithelium with moderate fibrocollagenous densities and multinucleated giant cells, occasional lymphocytes or neutrophils but no plasma cells were identified. This is the first series of this familial arthropathy with a triad of camptodactyly, arthropathy and coxa vara [CAC syndrome] in Saudi Arabia which is to be considered in patients where more than one family member has juvenile arthritis