Dermatomyositis in Six Patients Without Initial Muscle Involvement

Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis

“…Amyopathic dermatomyositis has been individualised in the spectrum of DPM [11,19]. In most patients, amyopathic dermatomyositis does not progress to myositis [20], a finding which may reflect the effect of early treatment [11], or indicating that it may be a distinct entity in the spectrum of DPM.…”

“…In the initial description of six cases [19], one patient had "pulmonary fibrosis", which persisted despite corticosteroid treatment. Lung involvement may be uncommon in amyopathic dermatomyositis, since no case of interstitial lung disease was reported in two recent series of 37 and 13 patients, respectively [20,21].…”

“…The time interval between respiratory and muscular or skin symptoms was clinically relevant, with a median delay of 100 and 340 days, respectively. When a NSIP pattern is found on lung biopsy, attention should be paid to muscular or cutaneous symptoms that may appear at some time during the course of the disease [11,19], particularly in patients with anti-tRNA-synthetase antibodies. Lung involvement may be frequently associated with cutaneous manifestations of DPM, since 13 of 17 of patients from the current study had skin manifestations, whereas dermatomyositis accounted for a minority of the cases in a large series of DPM [23].…”

Occult connective tissue diseases mimicking idiopathic interstitial pneumonias

“…In a meta-analysis of 291 cases of amyopathic DM [47], 13% of patients had ILD. In six patients with amyopathic DM, KRAIN [48] found two patients with pulmonary fibrosis in whom pulmonary fibrosis had contributed to delay in diagnosis. In a study involving 14 patients with ILD and amyopathic DM, SUDA et al [43] described two types of ILD, an acute/subacute type with a relatively high mortality and a chronic type.…”

“…The prevalence of ILD in amyopathic DM ranges 10-20% [47,48]. In a meta-analysis of 291 cases of amyopathic DM [47], 13% of patients had ILD.…”