Retropharyngeal edema: A rare manifestation of eosinophilic granulomatosis with polyangiitis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by excessive eosinophil accumulation in the peripheral blood and affected tissues with development of granulomatous vasculitic organ damage. Although upper airway and neck involvement is seen in patients with EGPA, retropharyngeal inflammation has never been reported. We report a case of retropharyngeal edema in a 70-year-old woman with EGPA. Her symptoms improved and the retropharyngeal edema disappeared on computed tomography following treatment. EGPA should be considered as a differential diagnosis in patients with asthma presenting with neck swelling and dysphagia.

Blue-collar work is a risk factor for developing IgG4-related disease of the biliary tract and pancreas.

BACKGROUND & AIMS: Immunoglobulin G4-related disease (IgG4-RD) of the biliary tract and pancreas is a fibroinflammatory disease of unknown origin with striking male predominance. We aimed to investigate whether blue-collar work and occupational contaminant exposure are risk factors for IgG4-RD of the biliary tract and pancreas. METHOD: We performed an age-/sex-matched case-control study in the largest academic medical centers of the Netherlands. Occupational history was surveyed by questionnaires. The International Standard Classification of Occupations (ISCO88) was used to classify jobs. Job exposure matrices ALOHA and DOM were utilized to assess the years individuals were exposed to compounds. The disease control cohort consisted of patients from 6 equally sized groups. Conditional logistic regression was used to assess effects of blue-collar work and exposure to occupational contaminants on developing IgG4-RD of the biliary tract and pancreas. RESULTS: Overall, 101 patients with IgG4-RD of the biliary tract and pancreas were matched 1:3 to 303 controls. Patients with IgG4-RD had a lower level of education (p = 0.001). Individuals who at least once performed blue-collar work (>1 year), had higher odds of developing IgG4-RD than individuals that only performed white-collar work (odds ratio [OR] 3.66; CI 2.18-6.13; p <0.0001). Being ever exposed (>1 year) to industrial ALOHA (e.g. mineral dust; vapors-dust-gases-fumes) and DOM compounds (e.g. asbestos) resulted in higher odds of IgG4-RD (OR 2.14; 95% CI 1.26-3.16; p <0.001 and OR 2.95; 95% CI 1.78-4.90; p <0.001, respectively). CONCLUSION: Blue-collar work is a risk factor for developing IgG4-RD of the biliary tract and pancreas putatively driven by exposure to selected industrial compounds; this may explain the striking male predominance among patients. LAY SUMMARY: Immunoglobulin G4-related disease (IgG4-RD) causes tumor-like lesions and typically affects middle-aged to elderly men. The background and cause of this disease remain relatively unknown. In this study, we identified blue-collar work as a risk factor for developing IgG4-RD of the biliary tract and pancreas, which may explain the striking male predominance among patients. Furthermore, these results suggest that toxic exposure to occupational contaminants may drive autoimmunity in IgG4-RD of the biliary tract and pancreas.

Immunoglobulin G4-Related Coronary Artery Aneurysm-Associated Stent Migration.

A 65-year-old woman with immunoglobulin G4-related disease (IgG4-RD), which was managed with a corticosteroid, underwent percutaneous coronary stent implantation in the left anterior descending artery as a result of angina pectoris. After 9 months, coronary angiography revealed stent migration and occlusion caused by progression of a coronary aneurysm potentially associated with IgG4-RD. (Level of Difficulty: Advanced.).

Epicardial Nodules as the Initial Manifestation of Immunoglobulin G4-Related Pericarditis.

Immunoglobulin G4 (IgG4)-related pericarditis, an immune-mediated fibro-inflammatory condition, is a rare yet life-threatening disease presenting with constrictive pericarditis. We describe a case of IgG4-related pericarditis presenting with epicardial nodules successfully treated with corticosteroids. This case highlights the clinical significance of assessing IgG4-related pericarditis in the diagnostic workup of pericardial masses. (Level of Difficulty: Advanced.).

Constrictive Pericarditis Caused by IgG4-Related Disease Requiring Pericardiectomy After Partial Response to Corticosteroids.

Immunoglobulin G4-related disease is a systemic fibroinflammatory disease; pericardial involvement has occasionally been reported in publications. A 79-year-old man with biopsy-proven immunoglobulin G4-related disease with pleural involvement was admitted in acute heart failure, with imaging and hemodynamic studies consistent with constrictive pericarditis. He was treated with corticosteroids for 2 months with partial response manifest by decreases in pericardial thickening and immunoglobulin G4 levels. However, persistent constriction required pericardiectomy, leading to significant symptomatic improvement. (Level of Difficulty: Intermediate.).

Immunoglobulin G4-Related Multiple Giant Coronary Artery Aneurysms and a Single Left Gastric Artery Aneurysm.

Coronary artery aneurysm (CAA) is potentially life-threatening. We describe a case of multiple giant CAAs and a single left gastric artery aneurysm caused by immunoglobulin G4-related disease (IgG4-RD). Our case highlights the significance of assessing IgG4-RD in the diagnosis of CAA and screening for other concurrent cardiovascular involvements. (Level of Difficulty: Intermediate.).