Rapidly progressive interstitial lung disease combined with pneumocystis jiroveci pneumonia in a patient with single anti-TIF-1γ antibody positive dermatomyositis in the context of an underlying tumor

BMC Pulmonary Medicine

Case Report

Hao Li¹  Haipeng Guo²  Jinfeng Yue²  Hongjun Lu³  Arezou Bikdeli⁴  Daqing Li⁴  Hengzhe Zhang⁵  Xiaohui Hou⁵ 

[1] Department of Hematology, Qilu Hospital, Shandong University, 250012, Jinan, Shandong, China;Department of Intensive Care Medicine, Qilu Hospital, Shandong University, 250012, Jinan, Shandong, China;Department of Intensive Care Medicine, Traditional Chinese Medicine of Rizhao Hospital, 276800, Rizhao, Shandong, China;Key Laboratory of Cardiovascular Remodeling and Function Research, Chinese Ministry of Education, Chinese National Health Commission and Chinese Academy of Medical Sciences, State and Shandong Province Joint Key Laboratory of Translational Cardiovascular Medicine, Department of Cardiology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, 250012, Jinan, Shandong, China;Key Laboratory of Cardiovascular Remodeling and Function Research, Chinese Ministry of Education, Chinese National Health Commission and Chinese Academy of Medical Sciences, State and Shandong Province Joint Key Laboratory of Translational Cardiovascular Medicine, Department of Cardiology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, 250012, Jinan, Shandong, China;Department of Intensive Care Medicine, Qilu Hospital, Shandong University, 250012, Jinan, Shandong, China;

关键词: Dermatomyositis;    Pneumocystis jiroveci pneumonia;    Interstitial lung disease;    Rapidly progressive interstitial lung disease;    Anti-transcriptional intermediate factors-1γ antibody;    Rapid weight loss;    Paraneoplastic syndrome;    Clinical case;   

DOI  :  10.1186/s12890-023-02542-0

 received in 2023-03-20, accepted in 2023-06-28,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundInterstitial lung disease (ILD) is a frequently observed comorbidity in autoimmune diseases such as dermatomyositis/polymyositis (DM/PM), and it is significantly associated with specific autoantibody types. One unique antibody type is the anti-transcription intermediate factor-1γ antibody (anti-TIF-1γ Ab), which has a positive rate of only 7%. It is often found in combination with malignancy and rarely with ILD, particularly rapidly progressive ILD (RPILD). In some cases, the presence of ILD in individuals with DM may indicate a paraneoplastic syndrome. Pneumocystis jiroveci pneumonia (PJP) typically occurs due to intensive immunosuppressive therapy, human immunodeficiency virus (HIV) infection, or malignancy, and rarely as an isolated condition.Case presentationA 52-year-old man with a history of rapid weight loss but non-HIV infected and not immunosuppressed who presented with fever, cough, dyspnea, weakness of the extremities, characteristic rash and mechanic's hand. Pathogenic tests suggested PJP, laboratory tests suggested a single anti-TIF-1γ Ab positive DM, imaging suggested ILD, and pathology revealed no malignancy. RPILD and acute respiratory distress syndrome (ARDS) developed after anti-infection and steroid hormone therapy. After mechanical support therapy such as Extracorporeal Membrane Oxygenation (ECMO), the patient developed late-onset cytomegalovirus pneumonia (CMVP), complicated bacterial infection, and ultimately death. Additionally, we discuss the potential causes of rapid weight loss, the mechanisms by which anti-TIF-1γ Ab may lead to ILD, and the possible connection between anti-TIF-1γ Ab positivity, rapid weight loss, immune abnormalities, and opportunistic infections.ConclusionsThis case emphasizes the importance of early recognition of malignant tumors and pulmonary lesions, assessment of the body's immune status, prompt initiation of immunosuppressive treatment, and prevention of opportunistic infections in individuals with single anti-TIF-1γ Ab positive DM presenting with rapid weight loss.

【 授权许可】

CC BY   
© The Author(s) 2023

【 图 表 】

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