Rapid and complete resolution of ascites and hydrothorax due to nephrotic syndrome caused by renal amyloidosis in a patient with juvenile chronic arthritis treated with adalimumab.

摘要

TNF-blocking agents have been reported to be successful in the treatment of various inflammatory disorders complicated with AA amyloidosis [1-9], and they are a promising therapeutic alternative to DMARDs and cytostatics. We report an excellent clinical response to treatment with adalimumab ina patient with AA amyloidosis complicating juvenile chronic arthritis.A 31-year-old woman, diagnosed at the age of 3 years with polyarticular-onset RF-negative ANA-positive juvenile chronic arthritis, was admitted to the hospital with mild dyspnoea, hypertension, poor general condition, and lower-limb oedema. She gave a 1-year history of gradually arising ascites and proteinuria up to 9.8 g/d prior to hospitalization. She also reported joint tenderness and swelling, and morning stiffness. At the age of 13 years she was diagnosed to have a nephrotic syndrome and secondary amyloidosis was confirmed with renal biopsy. Her medical history revealed that she had been treated with glucocorticosteroids, various disease modifying antirheumatic drugs (DMARDs) and chlorambucil.