1. Clinical presentation. This resembles the subacute form of the painful articular syndrome, but joint effusion is present. Indeed, it may likely be a continuum of the same process. Symptoms develop during a period of weeks and abate, usually within a month. There is a predilection for the lower extremities, and the pain may be quite severe, with direct distal femur tenderness. The joint fluid is typically not inflammatory (<10 3 cells per milliliter), although the patient may respond dramatically to intraarticular steroid. As an important distinction from classic presentations of reactive arthritis, these patients do not possess the HLA-B27 major histocompatibility complex (MHC) class I phenotype and do not demonstrate the extraarticular manifestations of Reiter's syndrome (i.e., conjunctivitis, urethritis).
2. Pathology. Although the joint fluid is not inflammatory, synovial biopsy in these patients usually shows some degree of mononuclear and plasma cell infiltrate. This is usually mild and seldom of the severity seen in other forms of inflammatory arthropathy. Periosteal reaction may be found on radiographs, and these patients may have hypertrophic osteoarthropathy. For this reason, it is prudent to exclude intercurrent infection, especially of a pulmonic source, in these patients. It is tempting to view this syndrome as part of a continuum of periarticular ischemia that produces the acute painful articular syndromes.
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