IgG4 Related Sclerosing Disease With Multiple Organ Involvements and Response to Corticosteroid Treatment

Abstract

Great attention has been drawn toward the recently defined IgG4 related sclerosing disease, an entity incorporating autoimmune pancreatitis and other organ involvements, hypergammaglobulinemia and deposition of IgG4 in affected tissues. We demonstrate an index case of this disease with multiple organ involvements and an excellent response to corticosteroid treatment. A 69-year-old white man was diagnosed with IgG4 related sclerosing disease with involvement of lungs, pancreas, submandibular glands, lymph nodes, and kidney. In addition, the patient had polyclonal IgG hypergammaglobulinemia and hypocomplementemia. A renal biopsy showed tubulointerstitial nephritis with extensive lymphoplasmacytic infiltrate. Biopsy of the lung showed "inflammatory pseudotumor" with lymphocyte and plasma cell infiltration. IgG4 positive plasma cells were seen in the biopsy of submandibular gland and the kidney. Immuno-staining of the renal biopsy specimen showed vascular deposition of complement split product C4d. Corticosteroid treatment of 2 months showed complete resolution of the disease process. We demonstrate deposition of C4d in the IgG4 related tubulointerstitial nephritis for the first time.

Infraorbital nerve swelling associated with autoimmune pancreatitis

“…5 Recently, AIP has been reported to be associated with various extrapancreatic lesions in organs such as lacrimal and salivary gland lesions, 6,7 hilar lymphadenopathy, 7,8 interstitial pneumonia, 7,9,10 sclerosing cholangitis, 7,11-14 renal lesions, 7,15,16 retroperitoneal fi brosis, 7,[17][18][19] and prostatic lesions. 7,[20][21][22] These pancreatic lesions and extrapancreatic lesions have been reported as IgG4-related sclerosing disease, 23 systemic IgG4-related plasmatic syndrome (SIPS), 24 and IgG4-positive multiorgan lymphoproliferative syndrome 25 because pathological fi ndings similar to those of AIP (i.e., abundant IgG4-positive plasma cells, lymphoplasmacytic infi ltration, fi brosis) were found. To the best of our knowledge, lesions of branches of the trigeminal nerve associated with AIP or infi ltrated by IgG4-positive plasma cells have not been reported in the Englishlanguage literature.…”

IgG4-related inflammatory pseudotumor of the kidney mimicking renal cell carcinoma: A case report

“…In the few previous reports of renal involvement with IgG4-RD, the renal lesions have been observed to decrease in size following corticosteroid therapy, with some disappearing and others recurring subsequent to cessation of treatment (10)(11)(12). Furthermore, corticosteroids have been reported to improve renal function and decease serum IgG4 levels after 1 month of therapy; however, renal function may not be totally recovered (10).…”

IgG4-related disease: a novel, important but easily missed condition

“…22 However, it has become clear that IgG4-RD is neither confined to Japan nor patients of Japanese origin but affects patients around the world. 3,[23][24][25][26][27][28][29] Epidemiological data come largely from a single Japanese study of AIP which described a male:female ratio of 2.85:1, peak age of onset of 61-65 years old and an estimated prevalence of 0.82 per 100,000 adults. 30 There is no reliable estimate of prevalence in the West, however it can be seen in comparison with other better known conditions (the prevalence of Hodgkin's lymphoma and amyloid A [AA] amyloidosis in Western Europe for example are 0.5 and 1.0 per 100,000 respectively).…”