IgG4-positive Pulmonary Disease

Abstract

We report herein high-resolution computed tomography findings from a patient with IgG4-related pulmonary disease for the first time. The 61-year-old male patient complained of low-grade fever, dry mouth, and night sweats. He was diagnosed as having autoimmune pancreatitis, Sjögren syndrome, and elevated serum IgG4. High-resolution computed tomography of the lungs showed dense alveolar consolidation and air bronchograms in bilateral perihilar regions. IgG4-positive lymphoplasmacytes were detected in pulmonary lesions by immunostaining of biopsy samples. IgG4-related pulmonary disease can be associated with various radiologic findings.

IgG4-related Lung Disease with Organizing Pneumonia Effectively Treated with Azathioprine

“…The combinations of steroids with cyclosporine, AZA, mycophenolate mofetil, rituximab, and MTX have also been found effective in patients with IgG4-RD (15,16,(20)(21)(22), with cyclosporine reported to be the only agent effective in treating pulmonary lesions (11). Furthermore, we were unable to find any reports discussing the characteristics of steroid-resistant IgG4-RLD.…”

“…However, the infiltrative type, as in our patient, has not been clearly described. There have been several case reports of patients with IgG4-RD exhibiting organizing pneumonia (8)(9)(10)(11)(12). However, pulmonary lesions have not been included in the diagnostic criteria for IgG4-RD (13).…”

Systemic immunoglobulin G4 (IgG4) disease and idiopathic orbital inflammation; removing ‘idiopathic’ from the nomenclature?

“…12,13 It was first reported in relation to autoimmune pancreatitis 14 and later the link with other organs was published, namely the liver, 15 salivary gland 16 and the retroperitoneal space. 17 Now, subtypes of interstitial lung disease 18 and tubulointerstitial nephritis 19 are now also considered part of the IgG4-related disease spectrum.…”

Immunoglobulin G4 (IgG4)-Positive or -Negative Ocular Adnexal Benign Lymphoid Lesions in Relation to Systemic Involvement

“…The entity includes autoimmune pancreatitis, sclerosing cholangitis, Mikulicz disease (chronic sclerosing dacryoadenitis with sialadenitis), [7][8][9][10][11] Küttner tumor (chronic sclerosing sialadenitis), 12 and retroperitoneal fibrosis. Systemic lymphadenopathy, 13,14 lung and liver inflammatory pseudotumors, 15 interstitial lung disease, [16][17][18] and tubulointerstitial nephritis [19][20][21] are also considered as part of IgG4-related disease.…”