Non-gouty arthritis in sickle cell disease: report of 37 consecutive cases.

Abstract

SUMMARY Arthritis in association with sickle cell disease was seen in 37 patients in a 2½/2-year period. Cases of gout and of avascular necrosis of the femoral head were excluded. In 12 patients a non-inflammatory effusion occurred during the course of a painful crisis, in 12 patients an ankle effusion occurred in association with spontaneous development or deterioration of leg ulceration, and in 13 patients there was a group of miscellaneous arthritides. Ankle arthritis with leg ulceration has not been previously recognised, and its association with spontaneous ulceration, which is presumed to have a vaso-occlusive origin, is compatible with ischaemic synovial damage. The aetiology may therefore be similar to that believed to account for effusions in association with the painful crisis.

Prevalence and Clinical Characteristics of Rheumatoid Arthritis in an Inner City Population with Sickle Cell Disease

“…The first study of rheumatic conditions in a large Jamaican cohort of sickle cell patients was conducted by de Ceulaer et al in 1984, including the review of 1100 SCD records in which the investigators reported four systemic lupus erythematosus patients, one RA, and two patients with chronic non-deforming polyarthritis [5]. In that study the prevalence of RA among SCD patients was 0.27% if non-deforming polyarthritis cases are included.…”

“…For example, in large Jamaican cohort of 1100 SCD patients reported by de Ceulaer et al in 1984, the prevalence of RA among SCD patients was estimated to be 0.27% if non-deforming polyarthritis cases are included [5]. The musculoskeletal manifestations of SCD including joint involvement might obscure evolving RA among these patients resulting in delayed diagnosis.…”

Rheumatological manifestations of haematological diseases.

“…Sympathetic joint effusion results from involved synovium or adjacent bone or marrow. Synovial fluid may be mildly inflammatory, and monoclonal and polyclonal antibodies have been used to identify early leukaemic antigens in the synovial fluid.48 4 However, normal synovial fluid has been found after mononuclear cell infiltration of synovial tissue has already occurred.50 Children do not appear to develop gout, even with high serum urate concentrations,5' but adults do, particularly during treatment. Radiological abnormalities include osteoporosis (fig 4) and osteolysis.…”

“…4 The joints feel warm and tender, and septic arthritis, acute gout, and haemarthrosis should be excluded. Joint aspirate is typically yellow, non-inflammatory, sterile, and free of urate crystals.…”

“…Ankle arthritis is occasionally associated with vaso-occlusive leg ulceration, implying that joint effusion is secondary to synovial and bone ischaemia or infarction. 4 Osteonecrosis contributes significantly to the disabling skeletal manifestations of sickle cell disease and by 2 years of age, up to 45% of children have dactylitis with recurrent, painful swelling.5 This 'hand-foot' syndrome is caused by bony infarction and digits are permanently shortened after epiphyseal plate damage. Sickle cell disease is probably the commonest cause of avascular necrosis world wide, with 150 000 estimated cases in Zaire alone.6 Most reports describe femoral head involvement (fig 1) with an incidence of 5-33% in large series;7 other sites include the humeral head (2-17%), 8 9 distal femur, calcaneum, and ribs.…”