IgG4 immune response in Churg–Strauss syndrome

Abstract

ObjectiveT-helper type 2 responses are crucial in Churg–Strauss syndrome (CSS) and may enhance the production of IgG4 antibodies. The authors assessed the IgG4 immune response in CSS patients.MethodsThe authors included 46 consecutive patients with CSS (24 with active and 22 with quiescent disease), 26 with granulomatosis with polyangiitis (GPA, Wegener's), 25 with atopic asthma and 20 healthy controls and determined serum IgG, IgM, IgA, IgE and IgG subclass levels. Tissue infiltration by IgG4 plasma cells was assessed in nine patients with CSS, 10 with GPA, 22 with chronic sinusitis (11 with and 11 without eosinophilia).ResultsIgG4 levels were markedly higher in active CSS patients than in controls (p<0.001 vs all control groups). Serum IgG4 correlated with the number of disease manifestations (r=0.52, p=0.01) and the Birmingham vasculitis activity score (r=0.64, p=0.001). Longitudinal analysis in 12 CSS cases showed that both the IgG4 level and IgG4/IgG ratio dropped during disease remission (p=3×10−5 and p=6×10−4, respectively). Tissue analysis did not show an increased IgG4 plasma cell infiltration in CSS biopsies compared with control groups.ConclusionsSerum IgG4 levels are markedly elevated in active CSS and correlate with the number of organ manifestations and disease activity.

IgG4-Related Sialoadenitis with a Skin Lesion and Multiple Mononeuropathies Suggesting Coexistent Cryoglobulinemic Vasculitis

“…We considered EGPA because of the history of asthma, mononeuropathy multiplex, eosinophilia and leukocytoclastic vasculitis. Additionally, high levels of serum IgG4 have been reported with EGPA (26). However, a negative result for ANCA and a lack of literature on concomitant cryoglobulinemia eliminated a diagnosis of EGPA.…”

“…Eosinophilia is a common feature of IgG4-RD (3) and an association between IgG4-RD and conditions like allergic rhinitis or asthma has been reported (12). In EGPA, an increase in IgG4-positive plasma cells is not as prominent (not more than 40% of the infiltrating IgG-positive plasma cells) (26), as it is in IgG4-RD.…”

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

“…Serum total IgE levels are elevated in most patients but they lack specificity for common allergens [45]. Recent data have shown that serum IgG4 levels were high in 75% of patients with active EGPA, better correlating with its activity than IgE [62]. ANCA may appear several years before the onset of vasculitis [63].…”

Ear, nose, and throat involvement in eosinophilic granulomatosis with polyangiitis

“…Salivary gland biopsy demonstrated vasculitis in one case and eosinophilic granulomas in the remaining two cases (27Á29). In some of these patients, elevated serum IgG4 levels and infiltration of IgG4-positive plasmacytes in the nasal mucosa were observed, which suggests some common pathogenic mechanisms between IgG4-related disease and EGPA (31,32).…”