Approximately 50% of idiopathic inflammatory myopathies (IIMs) are associated with interstitial lung disease (ILD). Typically, IIM-ILD manifests as nonspecific interstitial pneumonia. We herein report a rare case of a 78-year-old man with autoimmune pulmonary alveolar proteinosis (PAP) that developed during IIM treatment. The diagnosis of autoimmune PAP was based on detecting anti-granulocyte-macrophage colony-stimulating factor antibodies. We postulated that PAP may have been induced by IIM treatment with prednisolone. Our case suggests that the possibility of autoimmune PAP should be considered in patients with lung lesions during the clinical course of IIM.

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9970810PMC
http://dx.doi.org/10.2169/internalmedicine.9558-22DOI Listing

Publication Analysis

Top Keywords

autoimmune pulmonary
8
pulmonary alveolar
8
alveolar proteinosis
8
idiopathic inflammatory
8
iim treatment
8
autoimmune pap
8
autoimmune
4
proteinosis treatment
4
treatment idiopathic
4
inflammatory myopathy
4

Similar Publications

Interstitial lung disease (ILD) can lead to pulmonary hypertension (ILD-PH), worsening prognosis and increasing mortality. Diagnosing ILD-PH is challenging due to the limitations of imaging methods. Right heart catheterization (RHC) is the gold standard for diagnosing PH but is limited to ILD patients considered for lung transplantation.

View Article and Find Full Text PDF

Autoimmune pulmonary alveolar proteinosis (aPAP), which accounts for >90% of all cases of PAP, is a rare lung disease mediated by granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies that block GM-CSF signalling, leading to reduced surfactant clearance causing abnormal accumulation of alveolar surfactant and impaired gas exchange [1-3]. The current standard of care for aPAP is whole-lung lavage (WLL), which is invasive, resource intensive, carries procedural risk, does not address the underlying cause of disease and often must be repeated regularly [4]. Hence, there is a therapeutical need to address the underlying pathophysiology of the disease.

View Article and Find Full Text PDF

Abnormal autophagy regulation is implicated in lupus and other autoimmune diseases. We investigated autophagy in the murine pristane-induced lupus model. Pristane causes monocyte/macrophage-mediated endoplasmic reticulum (ER) stress in lung endothelial cells and diffuse alveolar hemorrhage (DAH) indistinguishable from DAH in lupus patients.

View Article and Find Full Text PDF

Background: There are limited studies of juvenile dermatomyositis (JDM) in low and middle-income countries (LMIC). Many demonstrate delays to care, high prevalence of severe manifestations, and high mortality. Given the disease-associated damage with JDM, understanding JDM in Africa further is critical.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!