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.

Histopathology of Lung Disease in the Connective Tissue Diseases

“…DAD is occasionally seen as a more acute manifestation of PM-DM lung disease in the clinical setting of acute exacerbation of underlying chronic lung disease, and it typically carries a poor prognosis similar to DAD associated with the clinical entity of acute respiratory distress syndrome. 22,33,36,37 Other more florid cellular manifestations of ILD, such as follicular bronchiolitis and lymphoid interstitial pneumonia (LIP) are rare in PM-DM. 1,7,34 Other rare manifestations of ILD associated with PM-DM include diffuse alveolar hemorrhage (DAH), pleuritis, airway involvement, and vasculitis.…”

The efficacy of calcineurin inhibitors for the treatment of interstitial lung disease associated with polymyositis/dermatomyositis

“…In the study by Douglas et al, 5 surgical lung biopsies were performed in 22 out of 58 patients with PM/DM-associated ILD, and organizing diffuse alveolar damage was observed in only two cases. On the other hand, this feature has been demonstrated in a number of autopsy cases of RPIP associated with CADM: 12,23 Presumably, its frequency in PM/DM is higher than that reported so far.…”

“…[2][3][4] Pulmonary complications are considered to be common determinants of morbidity and mortality in these patients. 3,[5][6][7][8][9][10][11][12] ILD associated with DM is frequently difficult to treat, being recognized as a more refractory disease compared with ILD associated with PM. 12,13 Certain patients who have the typical skin manifestations of DM in the absence of clinical manifestations of myositis are referred to as clinically amyopathic dermatomyositis (CADM).…”

“…3,[5][6][7][8][9][10][11][12] ILD associated with DM is frequently difficult to treat, being recognized as a more refractory disease compared with ILD associated with PM. 12,13 Certain patients who have the typical skin manifestations of DM in the absence of clinical manifestations of myositis are referred to as clinically amyopathic dermatomyositis (CADM). 14 The frequency of CADM is particularly elevated in Asian regions, and some patients with CADM develop rapidly progressive interstitial pneumonia (RPIP) with lethal respiratory failure.…”

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

“…When respiratory symptoms, image findings and/or the examinations listed above progress in less than several weeks or months, high‐dose GC (p.o. 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) . A blood trough level of 100–150 ng/mL for CsA and 5–10 ng/mL for Tac should be maintained unless renal damage is noted (evidence level III–V).…”