2016 Volume 49 Issue 10 Pages 661-668
New immunosuppressants and biologics have markedly changed the prognosis of patients with connective tissue diseases, such as rheumatoid arthritis and systemic lupus erythematosus, for whom there had previously not been any suitable treatments. However, there are still some refractory conditions that affect patients with connective tissue disease for which there are no effective treatments, such as progressive sclerosis of the skin and renal crisis in patients with systemic scleroderma (SSc) and rapidly progressive interstitial pneumonitis (RPIP) in clinical amyopathic dermatomyositis (CADM). Some studies have found that apheresis is an effective therapy for such refractory conditions; i.e., plasma exchange is effective against sclerosis of the skin and renal crisis in SSc, and polymyxin B-immobilized fiber column direct hemoperfusion combined with immunosuppressants is effective against RPIP in CADM. Moreover, a number of patients with rapidly progressive glomerulonephritis caused by vasculitis, such as those with microscopic polyangiitis or granulomatosis combined with polyangiitis, require hemodialysis treatment. Therefore, blood purification therapy is useful for treating refractory connective tissue diseases.