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ABSTRACT
Interstitial lung disease in polymyositis and dermatomyositis is a serious complication, associated with poor prognosis. In this article, we describe two cases with histological findings of organizing pneumonia (OP), based on transbronchial lung biopsy (TBB). One is a 66-year-old female patient with clinically amyopathic dermatomyositis (CADM) with anti-melanoma differentiation-associated gene 5 antibody, and another is a 61-year-old female patient with polymyositis with anti-Jo-1 antibody. Both of our cases rapidly deteriorated to death, and autopsy findings showed diffuse alveolar damage. Our experience indicates that TBB findings of OP may be a poor prognostic factor in CADM and polymyositis, in spite of the profile of myositis-specific antibodies.
Keywords: Clinically amyopathic dermatomyositis; dermatomyositis; diffuse alveolar damage; organizing pneumonia; polymyositis.
Interstitial lung disease (ILD) in polymyositis/ dermatomyositis (PM/DM) is associated with poor prognosis. Particularly, patients with clinically amyopathic dermatomyositis (CADM),1,2 more recently with anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody, often have rapidly progressive ILD (RP-ILD).3"5 Autopsy findings frequently reveal diffuse alveolar damage (DAD).6"8 On the other hand, cryptogenic organizing pneumonia (OP) is one of the idiopathic interstitial pneumonias (IIP),9,10 and presents as acute or subacute IIP.10 Chest computed tomography (CT) demonstrates patchy and often migratory consolidation, associated with ground"glass opacity. Cryptogenic OP often responds to corticosteroid treatment, leading to a favorable prognosis.9,11,12
Secondary OP associated with collagen vascular diseases had a poorer prognosis than cryptogenic OP.9,13 In this article, we described two cases with CADM and PM with OP findings, based on transbronchial biopsy (TBB) that subsequently developed DAD.
CASE REPORT
Case 1- A 66-year-old female patient was admitted with a two-week history of dry cough and exertional dyspnea. She also had photosensitivity and rashes on elbows for one year. Medical history included hysterectomy for endometrial cancer 17 years ago. Her oxygen saturation was 93% on room air. There were fine crackles in the lung bases. Manual muscle testing showed normal strength. Erythema was observed on the anterior chest, and the extensor surface of the right elbow (Gottron's sign). On laboratory test, ferritin level was highly elevated at 2110 ng/mL (<114), while creatine kinase (CK) level was normal. A level of KL-6 (Krebs von den Lungen 6), a serum marker for interstitial pneumonia,6 was normal. Anti-nuclear antibody and anti-Sjögren's syndrome antigen A (SSA/Ro) antibody were positive at...