Intensive immunosuppression versus prednisolone in the treatment of connective tissue diseases.

Intensive immunosuppression (IIS) was compared with prednisolone alone over a 2-year period in the treatment of severe connective tissue diseases. IIS consisted of 15 daily infusions of 750 mg antilymphocyte globulin (ALG), azathioprine 2.5 mg/kg/day, and prednisolone reducing from 150 mg, followed by maintenance azathioprine and prednisolone. The initial dose for prednisolone by itself was 60 mg and patients not responding to this regimen over a minimum of one month were then given IIS. Forty-one patients with life-threatening or severely disabling polyarteritis nodosa (PAN), dermatomyositis/polymyositis (DM), or systemic lupus erythematosus received one or other treatment. All 11 patients who received IIS for PAN remitted. Ten of these had renal impairment which was reversed or halted with IIS, and in 6 of these renal function had been deteriorating with prednisolone alone. One patient died of pneumonia in renal failure 9 months later but with PAN in remission. Two further patients, neither having renal involvement, achieved remission with prednisolone alone. Early cytotoxic treatment would seem to be indicated in PAN when there is renal involvement. Two patients with DM entered remission or prednisolone alone. The remaining 12, of whom 5 had failed steroid therapy, received IIS. Improvement or halting of deterioration was achieved in all 12 with best results in those without marked muscle wasting consequent to disease of long duration. The results suggest that IIS may be a useful adjunct in those patients failing to respond to prednisolone. IIS seemed no more effective than prednisolone alone in the treatment of the 14 patients with SLE and in particular lupus nephritis. Flares in disease activity were common in both groups and appeared to be related to prednisolone dosage. IIS was generally well tolerated, though infection occurred in 2 patients. Vertebral collapse or osteonecrosis of the femoral head occurred in 3 patients following IIS, all of whom had been previously receiving prednisolone for long periods.