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Prolonged remission in established rheumatoid arthritis (RA) is extremely rare. During a six year follow up of 458 patients Wolfe and Hawley1 observed two prolonged remissions of more than 48 months, and concluded that ‘once established, RA tends to remain, interrupted briefly in a small minority of patients by remission of disease.’ However prolonged remissions or cures have been described as secondary to Cushing’s disease2 and human immunodeficiency virus infection.3 4 We have observed a case of cure of RA accompanied by, and probably secondary to the development of γ heavy chain disease in the context of secondary Sjögren’s syndrome.5
RA was diagnosed in January 1986, in a 53 year old woman. Retrospective examination of her chart shows the presence of six of seven 1987 American Rheumatism Association revised criteria for the diagnosis of RA.6 Her treatment included intramuscular gold, non-steroid anti-inflammatory drugs, and several intra-articular injections of corticosteroids. Considerable improvement was noted at the end of 1988 and has persisted since. Gold injections (cumulative dose 2.4 g) were discontinued in March 1989 after discovery of proteinuria. The rheumatoid factor assay peaked at 1/1280 in May 1986 and was negative in April 1989. The sicca syndrome clinically attributed to secondary Sjögren’s syndrome, occurred in 1988 and has persisted since with enlargement of the salivary glands and the eyelids. Eyelid ‘fat’ was removed in August 1995 for cosmetic and functional purposes and showed infiltration by a monotonous population of small lymphocytes. There was decreased γ globulinaemia (4.8 g/l, normal value = 6 to 16 g/l) with free monoclonal γ heavy chains representing 75% of total IgG and an additional faint IgG κ band suggestive of a second monoclonal paraprotein. Flow cytometry of blood and bone marrow confirmed the presence of two B cell clones. The eyelid enlargement recurred quickly. Subsequent irradiation relieved the visual fields obstruction and was later successfully extended to the swollen submandibular regions. Sixteen months after diagnosis, the patient’s condition is stable.
The immunologically active polyclonal γ globulin concentration was 1 g/l (reference value 5.4 to 14.8), which is lower than in other leukaemias/lymphomas. The levels of IgA (0.38 g/l, reference value 0.65 to 1.48) and IgM (0.28 g/l, reference value 0.45 to 2.6) were also low. Lymphocyte count was 1120/μl with 54% of T cells and most of the B cells part of the clonal processes. Fifteen months later the paraprotein levels were stable, the polyclonal IgG have increased to 3 g/l, IgA to 0.55 g/l, IgM to 0.35 and lymphopenia worsened at 800.
We think that the following chain of events occurred in this case: immune disorder, autoimmune disease (RA and secondary Sjögren’s syndrome), malignant B cell neoplasm, secondary immunodeficiency with cure of the RA. The first three steps are well known, the fourth is probably exceptional but this case and others2 3 suggest that secondary immunodeficiency should be considered during spontaneous cures of well established RA.
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