hyper-IgG4 disease

The IMAD or ’hyper-IgG4 disease’ is a chronic inflammatory disease, characterized histologically by a lymphoplasmacytic inflammation with IgG4-positive cells and exuberant fibrosis, which leaves dense fibrosis on resolution.

Multifocal systemic fibrosclerosis (MSF) is a multisystem disease, which often mimics malignancy.

A typical example is idiopathic retroperitoneal fibrosis, but the initial report in 2001 was of sclerosing pancreatitis.

Synopsis

- idiopathic retroperitoneal fibrosis
- sclerosing pancreatitis
- inflammatory pseudotumor of the breast (15644785)

Synopsis

- multisystemic fibrosis

- IgG4 immune-complex tubulointerstitial nephritis (17895762)
- membranous glomerulopathy
- autoimmune hypophysitis (17954209)

- Mikulicz disease
- constrictive pericarditis (17727980)
- prostatitis
- interstitial pneumonia
- pulmonary vasculitis (17539856)
- lymphadenopathy with increased IgG4+ plasma cells
- inflammatory pseudotumors (17895762)

  • pulmonary inflammatory pseudotumor (16084938)
  • cervical epidural pseudotumor (10879775)
  • suprasellar pseudotumor (17954209)
  • bilateral diffuse retrobulbar pseudotumor (8512488)

- pachymeningitis and intracranial fibrosis (14606716)
- IgG4 periaortitis (18223321)

  • inflammatory abdominal aortic aneurysm (18223321)

Microscopical synopsis

- intense inflammatory cell infiltration with lymphocytes, plasma cells, scattered neutrophils
- eosinophilic aggregates
- venulitis
- obliterative arteritis
- majority of lymphocytes were T cells that expressed CD8 and CD4, with scattered B-cell-rich small lymphoid follicles
- significant increase in IgG4-positive plasma cells compared with controls

See also

- fever
- severe systemic disease
- retroperitoneal fibrosis
- IgG4-expressing plasma cells
- fibrotic diseases

Differential diagnosis

- inflammatory pseudotumour
- inflammatory myofibroblastic tumour
- plasma cell granuloma
- systemic fibrosis
- xanthofibrogranulomatosis
- multifocal fibrosclerosis

References

- Kasashima S, Zen Y, Kawashima A, Konishi K, Sasaki H, Endo M, Matsumoto Y, Kawakami K, Kasashima F, Moriya M, Kimura K, Ohtake H, Nakanuma Y. Inflammatory Abdominal Aortic Aneurysm: Close Relationship to IgG4-related Periaortitis. Am J Surg Pathol. 2008 Feb;32(2):197-204. PMID: 18223321

- Wong S, Lam WY, Wong WK, Lee KC. Hypophysitis presented as inflammatory pseudotumor in immunoglobulin G4-related systemic disease. Hum Pathol. 2007 Nov;38(11):1720-3. PMID: 17954209

- Cornell LD, Chicano SL, Deshpande V, Collins AB, Selig MK, Lauwers GY, Barisoni L, Colvin RB. Pseudotumors due to IgG4 immune-complex tubulointerstitial nephritis associated with autoimmune pancreatocentric disease. Am J Surg Pathol. 2007 Oct;31(10):1586-97. PMID: 17895762

- Cheuk W, Yuen HK, Chan JK.Chronic sclerosing dacryoadenitis: part of the spectrum of IgG4-related Sclerosing disease? Am J Surg Pathol. 2007 Apr;31(4):643-5. PMID: 17414116

- Kojima M, Sipos B, Klapper W, Frahm O, Knuth HC, Yanagisawa A, Zamboni G, Morohoshi T, Klöppel G. Autoimmune pancreatitis: frequency, IgG4 expression, and clonality of T and B cells. Am J Surg Pathol. 2007 Apr;31(4):521-8. PMID: 17414098

- Deshpande V, Chicano S, Finkelberg D, Selig MK, Mino-Kenudson M, Brugge WR, Colvin RB, Lauwers GY. Autoimmune pancreatitis: a systemic immune complex mediated disease.Am J Surg Pathol. 2006 Dec;30(12):1537-45. PMID: 17122509

- Watson SJ, Jenkins DA, Bellamy CO. Nephropathy in IgG4-related systemic disease. Am J Surg Pathol. 2006 Nov;30(11):1472-7. PMID: 17063091

- Cornell LD, Chicano SL, Deshpande V, Collins AB, Selig MK, Lauwers GY, Barisoni L, Colvin RB. Pseudotumors due to IgG4 Immune-Complex Tubulointerstitial Nephritis Associated With Autoimmune Pancreatocentric Disease. Am J Surg Pathol. 2007 Oct;31(10):1586-1597. PMID: 17895762

- Neild GH, Rodriguez-Justo M, Wall C, Connolly JO. Hyper-IgG4 disease: report and characterisation of a new disease. BMC Med. 2006 Oct 6;4:23. PMID: 17026742

- Taniguchi T, Kobayashi H, Fukui S, Ogura K, Saiga T, Okamoto M. A case of multifocal fibrosclerosis involving posterior mediastinal fibrosis, retroperitoneal fibrosis, and a left seminal vesicle with elevated serum IgG4. Hum Pathol. 2006 Sep;37(9):1237-9; PMID: 16938531

- Zen Y, Sawazaki A, Miyayama S, Notsumata K, Tanaka N, Nakanuma Y. A case of retroperitoneal and mediastinal fibrosis exhibiting elevated levels of IgG4 in the absence of sclerosing pancreatitis (autoimmune pancreatitis). Hum Pathol. 2006 Feb;37(2):239-43. PMID: 16426926

- Zen Y, Kitagawa S, Minato H, Kurumaya H, Katayanagi K, Masuda S, Niwa H, Fujimura M, Nakanuma Y. IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung. Hum Pathol. 2005 Jul;36(7):710-7. PMID: 16084938

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