Dermatomyositis in Six Patients Without Initial Muscle Involvement

Krain, Lawrence S.

doi:10.1001/archderm.1975.01630140099013

Cited by 94 publications

(32 citation statements)

References 3 publications

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“…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.…”

Section: Discussionmentioning

confidence: 99%

“…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].…”

Section: Discussionmentioning

confidence: 99%

“…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].…”

Section: Discussionmentioning

confidence: 99%

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Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis

et al. 2003

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis

et al. 2003

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“…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.…”

Section: Ild and Amyopathic Dermatomyositismentioning

confidence: 99%

“…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.…”

Section: Ild and Amyopathic Dermatomyositismentioning

confidence: 99%

Occult connective tissue diseases mimicking idiopathic interstitial pneumonias

Toya²,

2007

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In patients with interstitial lung disease (ILD), the diagnosis of idiopathic interstitial pneumonia is usually made after excluding, among other conditions, connective tissue diseases (CTDs). Although in most patients with a CTD and respiratory symptoms, the systemic nature of the disease is obvious, the ILD-related manifestations in CTDs may often dominate the clinical picture or precede systemic findings and thus mimic idiopathic interstitial pneumonia.With the exception of systemic lupus erythematosus, all CTDs may imitate chronic idiopathic interstitial pneumonias. In this setting, clues to an underlying CTD may be entirely absent or include subtle findings from various systems, including skin, vascular and musculoskeletal system or internal organs. Since nonspecific interstitial pneumonia is a relatively frequent histological pattern in CTDs, biopsy reports of nonspecific interstitial pneumonia should also prompt a search for an underlying CTD.Ultimately, diagnosis of a CTD requires confirmation with immunological testing; interpretation of the various laboratory tests should always be carried out in conjunction with clinical findings.The present article reviews specific clinical aspects of connective tissue disease-related interstitial lung disease that may help differentiate it from idiopathic interstitial pneumonia, especially when interstitial lung disease is the predominant or sole manifestation of an occult connective tissue disease.

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