Abstract
Recently, a new clinical entity, IgG4-positive multi-organ lymphoproliferative syndrome (IgG4+ MOLPS), characterized by hyper-IgG4 gammaglobulinemia and IgG4-positive plasma cell tissue infiltration, has been proposed. It includes autoimmune pancreatitis (AIP), Mikulicz’s disease, and many other inflammatory conditions affecting multiple organs. However, diagnosis is difficult if the disease is not suspected because serum IgG subclasses are not measured routinely and the affected organs vary. Because hypocomplementemia is often observed in this condition, we investigated the serum subclasses of IgG in patients with hypocomplementemia, especially of unknown etiology. We found 6 patients with high serum IgG4 levels among 10 patients with hypocomplementemia of unknown etiology who visited our hospital between December 2004 and September 2007. The results of additional pathological and imaging examinations in the 6 patients with high serum IgG4 levels were compatible with IgG4+ MOLPS. Our results suggest that hypocomplementemia of unknown etiology offers an opportunity to find cases of IgG4+ MOLPS.
Similar content being viewed by others
References
Kamisawa T (2006) IgG4-related sclerosing disease. Intern Med 45:125–126. doi:10.2169/internalmedicine.45.0137
Chari ST, Smyrk TC, Levy MJ et al (2006) Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol 4:1010–1016. doi:10.1016/j.cgh.2006.05.017
Hamano H, Kawa S, Horiuchi A et al (2001) High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 344:732–738. doi:10.1056/NEJM200103083441005
Yamamoto M, Takahashi H, Ohara M et al (2006) A new conceptualization for Mikulicz’s disease as an IgG4-related plasmacytic disease. Mod Rheumatol 16:335–340. doi:10.1007/s10165-006-0518-Y
Nakamura Y, Zen Y (2007) Pathology and immunopathology of immunoglobulin G4-related sclerosing cholangitis: the latest addition to the sclerosing cholangitis family. Hepatol Res Suppl 3:S478–S486
Kitagawa S, Zen Y, Harada K et al (2005) Abundant IgG4-positive plasma cell infiltration characterizes chronic sclerosing sialadenitis (Kuttner’s tumor). Am J Surg Pathol 29:783–791. doi:10.1097/01.pas.0000164031.59940.fc
Saeki T, Saito A, Hiura T et al (2006) Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4-related systemic disease. Intern Med 45:163–167. doi:10.2169/internalmedicine.45.1431
Saeki T, Saito A, Yamazaki H et al (2007) Tubulointerstitial nephritis associated with IgG4-related systemic disease. Clin Exp Nephrol 11:168–173. doi:10.1007/s10157-007-0464-9
Saeki T, Nishi S, Ito T et al (2007) Renal lesions in IgG4-related systemic disease. Intern Med 46:1365–1372. doi:10.2169/internalmedicine.46.0183
Masaki Y, Dong L, Kurose N et al. (2008) Proposal for a new clinical entity, IgG4-positive multi-organ lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis Aug 13 [Epub ahead of print]
Muraki T, Hamano H, Ochi Y et al (2006) Autoimmune pancreatitis and complement activation system. Pancreas 32:16–21. doi:10.1097/01.mpa.0000188308.75043.e4
Yamamoto M, Takahashi H, Naishiro Y et al (2008) Mikulicz’s disease and systemic IgG4-related plasmacytic syndrome (SIPS). Nihon Rinsho Meneki Gakkai Kaishi 31:1–8. doi:10.2177/jsci.31.1
Kuroki A, Shibata T, Honda H et al (2002) Glomerular and serum IgG subclasses in diffuse proliferative lupus nephritis, membranous lupus nephritis, and idiopathic membranous nephropathy. Intern Med 41:936–942. doi:10.2169/internalmedicine.41.936
Saeki T, Imai N, Ito T, Yamazaki H, Nishi S (2009) Membranous nephropathy associated with IgG4-related systemic disease and without autoimmune pancreatitis. Clin Nephrol 71(2):173–178
Lock RJ, Unsworth DJ (2003) Immunoglobulins and immunoglobulin subclass in the elderly. Ann Clin Biochem 40:143–148. doi:10.1258/000456303763046067
Takahashi N, Kawashima A, Fletcher JG, Chari ST (2007) Renal involvement in patients with autoimmune pancreatitis: CT and MR imaging findings. Radiology 242:791–801. doi:10.1148/radiol.2423060003
Cornell LD, Chicano SL, Deshpande V et al (2007) Pseudotumors due to IgG4 immune-complex tubulointerstitial nephritis associated with autoimmune pancreatocentric disease. Am J Surg Pathol 31:1586–1597
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Saeki, T., Ito, T., Yamazaki, H. et al. Hypocomplementemia of unknown etiology: an opportunity to find cases of IgG4-positive multi-organ lymphoproliferative syndrome. Rheumatol Int 30, 99–103 (2009). https://doi.org/10.1007/s00296-009-0925-4
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00296-009-0925-4