TABLE 1.
Immunoglobulin G4 (IgG4)-related lung disease reported in the literature*
| Author (ref), year | Total cases, n | Mean age at Dx, years | n, sex | Clinical features | Dx findings reported | Extrapulmonary involvement | Outcome |
| Matsui et al (12), 2012 | 25 | 61 | 18 M, 7 F | Cough (n=5), dyspnea (n=1) | IgG4/IgG positive cells >40%, elevated serum IgG4 levels, | AIP (n=3), nephritis (n=3), RPF (n=1), hypophysitis (n=1) | All responded to steroids, one relapse after 3 years |
| Matsui et al (13), 2012 | 18 | 62 | 14 M, 4 F | Mediastinal lymphadenopathy, thickening of perilymphatic hilum | High serum IgG4, biopsy with abundant IgG4+ plasma cell infiltration | AIP (n=12), CSS (n=9), RPF (n=3), lacrimal gland (n=5), bile duct (n=2), renal (n=3), prostate (n=3) | Good response to corticosteroids |
| Inoue et al (5), 2009 | 13 | 70 | 9 M, 4 F | Cough (n=7), fever (n=4), dyspnea (n=2) on exertion; solid nodules (n=4); GGOs (n=2), alveolar interstitial pattern (n=2), bronchovascular lesions (n=5) | Bx of involved organs showed lymphoplasmacytic infiltration, irregular fibrosis, obliterative vasculitis | AIP (n=3), CSS (n=3), periaortitis (n=2), renal (n=3), prostate (n=1) | NR |
| Yamashita et al (14), 2008 | 3 | 72 | 3 M | Dyspnea (n=1); pleural effusion (n=1); multifocal consolidation (n=1) | Lymphoplasmacytic infiltration, prominent vascular involvement, IgG4/IgG >40% | CSS (1), lacrimal gland (1) | Resolved with steroid (n=1); resected with no recurrence; diuresed with no recurrence |
| Suzuki et al, (15), 2011 | 2 | NR | NR | NR | NR | Pericardial effusion (n=1), CSS (n=1), RPF (n=1) | Steroids effective; complicated by NTM |
| Single case reports (16–30) | 17 | 63 Others NR | 10 M, 2 F Others NR | Dry cough, dyspnea, fever; atopic asthma; lung mass | Most with biopsy proven organ involvement with lymphoplasmacytic infiltration | Ocular inflammation, acquired hemophilia A, pericarditis, lacrimal gland, CSS, renal, prostate | Prednisone generally effective |
| Total | 78 |
*
Search criteria: Pubmed and Google scholar. (“immunoglobulin g”[MeSH Terms] OR “immunoglobulin g”[All Fields] OR “igg4”[All Fields]) AND related[All Fields] AND (“disease”[MeSH Terms] OR “disease”[All Fields]) AND (“lung”[MeSH Terms] OR “lung”[All Fields]). AIP Autoimmune pancreatitis; Bx Biopsy; CSS Chronic sclerosing sialadenitis; Dx Diagnosis; F Female; GGO Ground-glass opacities; M Male; NR Not reported; NTM Nontuberculous mycobacterium; ref Reference; RPF Retroperitoneal fibrosis