Diffuse alveolar damage in patients with dermatomyositis: a six-case series

Abstract

The clinical course of diffuse alveolar damage (DAD) was studied in six consecutive cases of dermatomyositis (DM) based on our hospital records over 8 years. Three patients had severe myopathy at presentation, and the other three patients showed clinically amyopathic DM (CADM). Interstitial pneumonia in all patients developed shortly after they manifested DM. DAD in five deceased patients, which was proven pathologically, did not respond to steroid therapy combined with cyclosporine or tacrolimus. Of these, two patients began receiving combination therapy before suffering respiratory symptoms, and one of them had elevated serum Krebs von der Lungen-6 (KL-6) levels before visible abnormalities appeared on a plain chest X-ray. Only one patient with CADM survived; this patient received intravenously administered pulse cyclophosphamide (IVCY) therapy intravenously for DAD from the early stage. Delayed adjunctive IVCY was ineffective for progressed DAD in the remaining five patients. Elevated serum ferritin levels were observed in all four patients examined and might have predicted the lethal DAD, as in a previous report. In conclusion, promptly beginning IVCY therapy may be beneficial for patients with DM and interstitial pneumonia who show elevated serum levels of ferritin or KL-6 with minimal pulmonary abnormalities.

Effects of Direct Hemoperfusion with Polymyxin B-immobilized Fiber on Rapidly Progressive Interstitial Lung Diseases

“…Pathologically, DAD is also evident in rapidly progressive ILD associated with ADM (15,16). A novel autoantibody, anti-CADM-140, was identified and patients with the antibody frequently had ADM with rapidly progressive ILD (17).…”

Treatment consensus for management of polymyositis and dermatomyositis among rheumatologists, neurologists and dermatologists

“…administration of prednisolone 1 mg/kg bodyweight per day with or without methylprednisolone pulse therapy) together with a calcineurin inhibitor should be started (evidence level III-V). 28,79,81,[161][162][163][164][165] A blood trough level of 100-150 ng/mL for CsA 81,162 and 5-10 ng/mL for Tac 164 should be maintained unless renal damage is noted (evidence level III-V). Addition of intermittent i.v.…”

“…Addition of intermittent i.v. CPA therapy (evidence level III-V) 81,[164][165][166] and/or IVIg (evidence level V) 28,167 should be attempted for serious or intractable cases. There are reports that high-dose GC therapy in combination with intermittent CPA therapy and cyclosporin was effective in treating rapidly progressive ILD with CADM (evidence level IV).…”

Immunosuppression for Connective Tissue Disease–Related Pulmonary Disease

“…Both drugs have been reported in case studies and small case series to be effective in the treatment of ILD associated with either polymyositis or dermatomyosistis. [62][63][64][65][66][67][68][69] The largest of these studies reports 15 cases (identified from a registry of 179 patients with myositis) of IIM-associated ILD treated with calcineurin inhibitors for a median of 24 months. The majority (11 cases) were being treated for refractory disease.…”