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Membranoproliferative Glomerulonephritis Type II (Dense Deposit Disease): An Update

Gerald B. Appel^*, H. Terence Cook, Gregory Hageman, J. Charles Jennette, Michael Kashgarian^||, Michael Kirschfink^¶, John D. Lambris^#, Lynne Lanning^**, Hans U. Lutz, Seppo Meri, Noel R. Rose, David J. Salant^||||, Sanjeev Sethi^¶¶, Richard J.H. Smith^##, William Smoyer^***, Hope F. Tully, Sean P. Tully, Patrick Walker, Michael Welsh, Reinhard Würzner^|||||| and Peter F. Zipfel^¶¶¶

^* Columbia University Department of Nephrology, New York, New York; Division of Investigative Science, Imperial College Faculty of Medicine, London, England; Department of Ophthalmology and Visual Sciences, University of Iowa, Carver College of Medicine, Iowa City, Iowa; Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, North Carolina; ^|| Department of Pathology, Yale University School of Medicine, New Haven, Connecticut; ^¶ Institute of Immunology, University of Heidelberg, Heidelberg, Germany; ^# Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; ^** Kidneeds, Iowa City, Iowa; Institute of Biochemistry, Swiss Federal Institute of Technology, Zurich, Switzerland; Department of Bacteriology and Immunology, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland; Center for Autoimmune Disease Research, Johns Hopkins School of Medicine, Baltimore, Maryland; ^|||| Department of Medicine, Boston University Medical Center, Boston, Massachusetts; ^¶¶ Department of Pathology, University of Iowa, Carver ^## Department of Otolaryngology, University of Iowa Carver College of Medicine, Iowa City, Iowa; ^*** Pediatric Nephrology Division, University of Michigan, Ann Arbor, Michigan; Milagros Research Fund, Chappaqua, New York, New York; Nephropathology Associates, Little Rock, Arkansas; Department of Internal Medicine, University of Iowa Carver College of Medicine, Iowa City, Iowa; ^|||||| Department of Hygiene, Microbiology and Social Medicine, Innsbruck Medical University, Innsbruck, Austria; and ^¶¶¶ Hans Knoell Institute for Natural Products Research, Jena, Germany

Address correspondence to: Dr. Richard J.H. Smith, Department of Otolaryngology, 200 Hawkins Drive, 21151 PFP, The University of Iowa, Iowa City, IA 52242. Phone: 319-356-3612; Fax: 319-356-4108; richard-smith{at}uiowa.edu

Membranoproliferative glomerulonephritis type II (MPGN II) is a rare disease characterized by the deposition of abnormal electron-dense material within the glomerular basement membrane of the kidney and often within Bruch’s membrane in the eye. The diagnosis is made in most patients between the ages of 5 and 15 yr, and within 10 yr, approximately half progress to end-stage renal disease, occasionally with the late comorbidity of visual impairment. The pathophysiologic basis of MPGN II is associated with the uncontrolled systemic activation of the alternative pathway (AP) of the complement cascade. In most patients, loss of complement regulation is caused by C3 nephritic factor, an autoantibody directed against the C3 convertase of the AP, but in some patients, mutations in the factor H gene have been identified. For the latter patients, plasma replacement therapy prevents renal failure, but for the majority of patients, there is no proven effective treatment. The disease recurs in virtually all renal allografts, and a high percentage of these ultimately fail. The development of molecular diagnostic tools and new therapies directed at controlling the AP of the complement cascade either locally in the kidney or at the systemic level may lead to effective treatments for MPGN II.

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