Alkylating cytostatic treatment in renal amyloidosis secondary to rheumatic disease.

Abstract

SUMMARY Fourteen consecutive patients with chronic inflammatory rheumatic disease and reactive renal amyloidosis were treated with alkylating cytostatics in 22 separate periods varying in duration between six and 30 months. Chlorambucil alone was given in 14 treatment periods, cyclophosphamide alone in six, and both alternately in two. The dosage was adjusted to attain a major suppression of the rheumatic inflammation and a blood lymphocyte level below 1-0 x 109/l.Renal function improved in 12 treatment periods, renal deterioration was arrested in three periods, and in another four periods the rate of functional decline slowed down. In the remaining three treatment periods, associated with further deterioration in renal function, treatment was inadequate owing to blood dyscrasia and failure to control hypertension. Glomerular filtration rate (GFR) was followed more closely in 10 treatment periods, in all of which the falling trend was arrested or reduced. The survival rate at five years was 93%. Three patients who dropped out of the treatment programme are so far the only ones not still alive. Nine are still being followed up after 6-17 years, and the other two remaining live patients have had renal transplants for five years.

Regression of the nephrotic syndrome in rheumatoid arthritis and amyloidosis treated with azathioprine

“…Four of these patients whose rheumatic disease responded to immunosuppressive therapy with either methotrexate or cyclophosphamide had a marked reduction or complete resolution of proteinuria (15). Berglund et a1 (16) recently updated the data on the 16 RA patients treated with alkylating cytostatic drugs in their 1987 study (14). Patients were treated with chlorambucil when they had active arthritis and an increase in serum creatinine levels or proteinuria.…”

“…Fourteen patients with rheumatic diseases and amyloidosis were treated with both chlorambucil and cyclophosphamide, but the 2 drugs were not directly compared (14). Nine of the patients had RA, 4 had ankylosing spondylitis, and 1 had JRA.…”

“…Nine of the patients had RA, 4 had ankylosing spondylitis, and 1 had JRA. In 19 of the 22 treatment periods, renal amyloidosis was considered "satisfactory or somewhat improved" (14).…”

BOOP (Bronchiolitis Obliterans Organizing Pneumonia) and Renal Amyloidosis Secon-dary to Infected Cystic Bronchiectasis

“…Çeşitli immünsupresif ilaçlar da sekonder amiloidoz tedavisinde denenmiştir. Bunlar içinde en eski ve en iyi bilineni klorambusil'dir (14). Sekonder amiloidozda etkili bulunan diğer ajanlar arasında kolşisin, siklosporin A ve diğer alkilleyici ajanların olduğu bildirilmekle birlikte tek bir çalışma dışında, prospektif, kontrollü ve randomize çalış-malar yoktur (15,16 …”

Long-term Follow-up of Systemic Reactive AA Amyloidosis Secondary to Rheumatoid Arthritis: Successful Treatment with Intermediate-dose Corticosteroid.

“…The effects of these drugs are, however, usually insufficient to suppress the progression of organ dysfunction after the appearance of symptomsdue to reactive AAamyloidosis. In the last decade several reports have advocated using immunosuppressants or cytostatics, including cyclophosphamide, chlorambucil, azathioprine and methotrexate, for treatment of reactive AAamyloidosis secondary to either RAor Still's disease (15)(16)(17)(18)(19)(20). These drugs can be expected to achieve an improvementin such symptomsas the nephrotic syndrome due to the intense suppression of the production of SAA,but their side effects might induce a worsening of organ failure in patients with reactive AAamyloidosis.…”