2001;344:732C8. A recent study indicates that Treg cells can suppress allergy in a phenomenon known as immune deviation. Normally, Treg cell response is directed against T cell epitopes of the respective antigen, suppressing antigen-specific T cell proliferation and reducing the production of Th-1- and Th-2-type cytokines. In IgG4RLD, overexpression of interleukin-10 and transforming growth factor- have been suggested to play an important role in skewing the immune response toward a fibroproliferative state (11). A review of the literature (Table 1) identifies a MK-5108 (VX-689) number of case reports and case series that have described the many facets of this disease, although there is definitely yet insufficient info to comment on the diseases epidemiology. Similarly, there is a paucity of info on management and prognosis of individuals affected by IgG4RLD. Because the manifestation and severity of IgG4RLD are disparate and protean, management is definitely guided by patient symptoms and physiological impairment of organs. Typically, systemic corticosteroids are the first line of treatment. Although there is no universal consensus within the dose or the period, MK-5108 (VX-689) experts suggest 0.5 mg/kg/day to 1 1 mg/kg/day for two to four weeks and then in tapering doses over three months. As LEG2 antibody illustrated by our instances, most instances of IgG4RLD are partially or totally responsive to corticosteroids, although the rates of recurrence are high following their discontinuation. In nonresponsive or regularly repeating instances, additional immunosuppressives are suggested, as illustrated in case 1. However, none of these therapies, including corticosteroids, has been validated in randomized controlled trials. More recently, some have used rituximab to deplete B cells and have reported superb response rates (1). The part of surgery is definitely uncertain. In instances of isolated lung lesions refractory to immunosuppressive therapy, a medical option may be regarded as if individuals possess significant symptoms and/or demonstrate significant organ impairment. Long term studies will further inform our understanding of the pathogenesis, natural progression, treatment and prognosis. TABLE 1 Immunoglobulin G4 (IgG4)-related lung disease reported in the literature* Author (ref), yearTotal instances, nMean age at Dx, yearsn, sexClinical featuresDx findings reportedExtrapulmonary involvementOutcomeMatsui et al (12), 2012256118 M, 7 FCough (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 yearsMatsui et al (13), 2012186214 M, 4 FMediastinal lymphadenopathy, thickening of perilymphatic hilumHigh serum IgG4, biopsy with abundant IgG4+ plasma cell infiltrationAIP (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 corticosteroidsInoue et al (5), 200913709 M, 4 FCough (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 vasculitisAIP (n=3), CSS (n=3), periaortitis (n=2), renal (n=3), prostate (n=1)NRYamashita et al (14), 20083723 MDyspnea (n=1); pleural effusion MK-5108 (VX-689) (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 recurrenceSuzuki et al, (15), 20112NRNRNRNRPericardial effusion (n=1), CSS (n=1), RPF (n=1)Steroids effective; complicated by NTMSingle case reports (16C30)1763 Others NR10 M, 2 F Others NRDry cough, dyspnea, fever; atopic asthma; lung massMost with biopsy verified organ involvement with lymphoplasmacytic infiltrationOcular swelling, acquired hemophilia A, pericarditis, lacrimal gland, CSS, renal, prostatePrednisone generally effectiveTotal78 Open in a separate window *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 Analysis; F Female; GGO Ground-glass opacities; M Male; NR Not reported; NTM Nontuberculous mycobacterium; ref Research; RPF Retroperitoneal fibrosis Post-test What are the characteristic histological features of IgG4RLD? IgG4RLD is definitely characterized by dense infiltration of IgG4-positive plasma cells and lymphocytes. In more severe instances, storiform fibrosis and obliterative.