Abstract
Background: IgG4-Related Disease(IgG4 RD)is a rare immune-mediated disease with multi-organ involvement.
Aim: To describe pulmonary manifestations of patients with IgG4-RD.
Methods: Chart review was performed of consecutive patients diagnosed with IgG4 RD between 2009-2014 at the PD Hinduja National Hospital,Mumbai.Included patients fulfilled diagnostic criteria of 'Disease Ministry of Health,Labor and Welfare,Japan.'
Results: 8 of 11 patients with IgG4 RD had pulmonary manifestations.Age of presentation ranged from 15-73 years.6 patients were female.Average delay in diagnosis was 9 months.Prior to diagnosis,3 patients received empiric antituberculous drugs and bronchoalveolar lavage performed in 3 patients was non-diagnostic.The right mainstem bronchus was found to be narrowed in 1 patient..Median IgG4 level was 4.76 mg/dl(IQR 3.26,6.1).Presenting symptoms,thoracic CT and hisptopathological findings are summarized
| Feature(no of patients) | |
| Symptoms | Fever(6) Cough(5) Shortness of breath(5) Weight loss(4) Stridor(1) |
| Thoracic CT findings(8 of 8 pts) | multiple small nodules< 1cm with an upper lobe predominance(5) ill defined patchy areas of consolidation(4) cervical and mediastinal homogeneously enhancing lymphadenopathy(4) ground glass opacities (3) peribronchial wall thickening(2) interlobular septal thickening(2) subglottic stenosis(2) mass-like lesion(1) bronchial stenosis(1) |
| Histopathological findings(4 of 8 pts) | subglottic biopsy(2)-non-diagnostic.Transbronchial lung biopsy(1)-Minimal interstitial fibrosis. Lymph node biopsy(1)-minimal inflammation and mild fibrosis. |
Conclusion: Pulmonary involvement in IgG4 RD is common and should be sought for in patients diagnosed with the disease.
- Copyright ©ERS 2015
