IgG4-related lung disease progressing to respiratory failure.
Yusuke KunimatsuNozomi TaniIzumi SatoYuri OguraKazuki HiroseTakayuki TakedaPublished in: Respirology case reports (2020)
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a systemic immune-mediated, fibroinflammatory disorder categorized as proliferative or fibrotic depending on the responsiveness of corticosteroid treatment. IgG4-related lung disease (IgG4-RLD) accounts for 13-14% of IgG4-RD cases, but respiratory failure is quite rare. A 71-year-old man diagnosed with interstitial lung disease was referred to our department after a 10-month observational period. He presented with respiratory failure at the first visit, with significant elevations in serum IgG4 levels and histopathological findings meeting the criteria of IgG4-positive plasma cells and IgG4/IgG-positive plasma cell ratio in transbronchial lung biopsy and inguinal lymph node biopsy, resulting in a diagnosis of IgG4-RD. Systemic corticosteroid treatment promptly ameliorated the respiratory failure. 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography showed significant FDG accumulation in the lung fields, indicating the proliferative and reversible status of IgG4-RLD, which responded well to corticosteroid treatment. The patient recovered from respiratory failure even after a 10-month observational period.
Keyphrases
- respiratory failure
- positron emission tomography
- computed tomography
- extracorporeal membrane oxygenation
- mechanical ventilation
- pet ct
- lymph node
- systemic sclerosis
- interstitial lung disease
- acute respiratory distress syndrome
- pet imaging
- magnetic resonance imaging
- ultrasound guided
- cell cycle arrest
- radiation therapy
- magnetic resonance
- cross sectional
- prostate cancer
- contrast enhanced
- cell proliferation
- idiopathic pulmonary fibrosis
- oxidative stress
- signaling pathway
- fine needle aspiration
- rectal cancer