A case of anti-melanoma differentiation-associated gene 5 antibody-positive interstitial lung disease complicated with tracheobronchial ulcers

Abstract

We herein report the first case, to our knowledge, of tracheobronchial ulcer with anti-melanoma differentiation-associated gene 5 (anti-MDA 5) antibody-positive interstitial lung disease (ILD). A 53-year-old man complained of shoulder and wrist pain and was suspected of having polymyalgia rheumatica at another hospital. Thereafter, treatment with prednisolone was started. Although his arthralgia improved, he suffered from progressive dyspnea on exertion and an abnormal shadow was noted on chest X-ray, so he was transferred to our hospital. Chest computed tomography scan revealed ground-glass opacities and intralobular septal thickening. We diagnosed him as having clinically amyopathic dermatomyositis associated with ILD based on the specific skin findings and elevated anti-MDA 5 antibody titer. Fiberoptic bronchoscopy showed ulcerations of the trachea and bronchus. Treatment with dose increments of prednisolone combined with other immunosuppressive drugs resulted in improvement of his respiratory condition and tracheobronchial lesions. Clinicians should be aware that tracheobronchial ulcers can be associated with anti-MDA 5 antibody-positive interstitial lung disease.

Anti-PL-7 antibody-positive dermatomyositis with progressive interstitial pneumonia complicated with tracheal ulcer

“…Tracheobronchial ulcers are rare extramuscular manifestations in IIM. We recently reported two cases of ARS antibody-positive interstitial pneumonia and MDA5 antibody-positive interstitial pneumonia associated with tracheobronchial ulcers [ 9 , 10 ]. Here, we present 5 cases of tracheobronchial ulcers in IIM together with other previously reported cases ( Table 1 ) [ [7] , [8] , [9] , [10] ].…”

“…We recently reported two cases of ARS antibody-positive interstitial pneumonia and MDA5 antibody-positive interstitial pneumonia associated with tracheobronchial ulcers [ 9 , 10 ]. Here, we present 5 cases of tracheobronchial ulcers in IIM together with other previously reported cases ( Table 1 ) [ [7] , [8] , [9] , [10] ]. All cases were associated with interstitial pneumonia, and 4 showed the subacute progressive course of interstitial pneumonia except for 1 case in which there was no detailed description of the clinical course.…”

“…There are few reports of tracheobronchial lesions as extramuscular manifestations of IIMs, and their characteristics and relevance to the IIMs are not clear [ [7] , [8] , [9] , [10] ]. We report a Case of DM with progressive interstitial pneumonia complicated by a tracheal ulcer.…”

Tracheal Ulcers Associated with Anti-synthetase Syndrome

“…The histopathology of these lesions revealed necrotizing tracheal inflammation (Picture 2B). Only two reported cases of DM have been associated with tracheobronchial ulceration (1,2). We recently described the first case of anti-melanoma differentiation-associated gene 5 antibody-positive interstitial pneumonia with a complaint of tracheobronchial ulcers (2).…”

“…Only two reported cases of DM have been associated with tracheobronchial ulceration (1,2). We recently described the first case of anti-melanoma differentiation-associated gene 5 antibody-positive interstitial pneumonia with a complaint of tracheobronchial ulcers (2). In the present case as well, the trachea ulcer might have been caused by a focal ischemic condition, such as Raynaud's phenomenon.…”