Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis

Abstract

This study investigated interstitial pneumonia associated with amyopathic dermatomyositis, dermatomyositis and polymyositis, paying particular attention to muscular and/or cutaneous manifestations and their chronology relative to lung involvement. Patients included four males and 13 females, aged 51.7±10.8 yrs, who had surgical lung biopsy.Diagnoses included dermatomyositis (10 patients), polymyositis (four patients) and amyopathic dermatomyositis (three patients). Solitary respiratory manifestations preceded the onset of any skin or muscle disease in four cases (24%). Reticular and ground glass opacities were the most frequent computed tomography (CT) findings. Pathological review showed nonspecific interstitial pneumonia (eleven, 65%; cellular, two; cellular and fibrotic, five; fibrotic, four), usual interstitial pneumonia (two), organising pneumonia (two), lymphocytic interstitial pneumonia (one), and unclassifiable interstitial pneumonia (one). Nonspecific interstitial pneumonia was the most common histological pattern of interstitial pneumonia in patients with amyopathic dermatomyositis (three of three) and in patients with respiratory symptoms as the initial clinical manifestation of the connective tissue disease (three of four). Survival at 5 yrs was 50%.This study shows the clinician should remain alert to potential muscular or cutaneous manifestations whenever a pathological diagnosis of nonspecific interstitial pneumonia is made.

Interstitial lung diseases associated with amyopathic dermatomyositis

“…Regarding the anti-Jo-1 antibody, only one patient with chronic ILD-ADM had this antibody among the present ILD-ADM study. To date, several studies have reported a low incidence of anti-Jo-1 antibody in ILD-ADM patients [11][12][13][17][18][19]. The current results were consistent with those studies.…”

“…These data suggest that chronic ILD is unlikely to pre-exist in the study patients with acute/subacute ILD. Taken together, these results may account for the contradictory data previously reported in the prognosis of ILD-ADM. Namely, the fatal progressive ILD-ADM described is likely to correspond to the acute/subacute ILD-ADM in the present study, while the ILD-ADM with a favourable prognosis reported by COTTIN et al [13] may be equivalent to chronic ILD-ADM. Although ethnic differences may affect the clinical manifestations of ILD-ADM, it should be noted that ILD-ADM has these two different forms.…”

“…These patients were often resistant to intensive therapy, such as highdose corticosteroids plus immunosuppressive agents, resulting in fatal respiratory failure. In contrast, a recent report from Europe emphasised a favourable prognosis of ILD associated with ADM (ILD-ADM) among ILD associated with ADM, DM, and polymyositis (PM) [13]. As ILD-ADM is a rare condition, its characteristics have not been fully clarified.…”

“…Previous studies, mainly from Asia, have demonstrated that ILD-ADM generally runs an aggressive course, leading to fatal respiratory failure [7][8][9][10][11][12]. In contrast, COTTIN et al [13] recently described a benign form of ILD-ADM. They reported three patients with chronic ILD-ADM, and corticosteroids alone achieved a favourable response in two.…”

“…As ADM is a rare disease, previous studies of ILD-ADM included only one, or a few patients [7][8][9][10][11][12][13]. The present study investigated the highest number of patients with ILD-ADM so far.…”