Bullous Pemphigoid With a Dual Pattern of Glomerular Immune Complex Disease
| Autor: | Marcel F. Jonkman, Jan J. Weening, Hendri H. Pas, Mahdi Salih, Tiina Hurskainen, Ewout J. Hoorn, Noor E. Taams, Marco W.J. Schreurs |
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| Přispěvatelé: | Internal Medicine, Pathology, Immunology, Translational Immunology Groningen (TRIGR), Microbes in Health and Disease (MHD) |
| Jazyk: | angličtina |
| Rok vydání: | 2016 |
| Předmět: |
Male
0301 basic medicine Pathology medicine.medical_specialty NEPHROPATHY DIAGNOSIS BLISTERS PATIENT Mice 030207 dermatology & venereal diseases 03 medical and health sciences Type IV collagen Glomerulonephritis 0302 clinical medicine Membranous nephropathy renal biopsy Pemphigoid Bullous glomerular basement membrane (GBM) medicine Animals Humans Immune Complex Diseases type XVII collagen anti-GBM disease Aged Mice Knockout Basement membrane medicine.diagnostic_test integumentary system business.industry Bullous pemphigoid nephrotic syndrome Glomerular basement membrane membranous nephropathy medicine.disease COLLAGEN 030104 developmental biology medicine.anatomical_structure Nephrology Renal biopsy business Immune complex disease autoantibody 180-kDa BP antigen |
| Zdroj: | American Journal of Kidney Diseases, 67(2), 302-306. W.B. Saunders American Journal of Kidney Diseases, 67(2), 302-306. W B SAUNDERS CO-ELSEVIER INC |
| ISSN: | 0272-6386 |
| Popis: | A 75-year-old man presented with a blistering skin disease and nephrotic syndrome. Bullous pemphigoid was diagnosed by linear immunoglobulin G (IgG) and C3 staining along the basement membrane zone of a skin biopsy specimen and by the presence of circulating IgG recognizing the 180-kDa bullous pemphigoid antigen (BP180; type XVII collagen). A kidney biopsy specimen showed endocapillary inflammation without crescents. Direct immunofluorescence showed strong IgG and C3 staining in a combined granular and linear pattern along the glomerular basement membrane. Electron microscopy showed subepithelial deposits. In serum, no antibodies against the Goodpasture antigen (type IV collagen) or phospholipase A(2) receptor were detected. Indirect immunofluorescence studies using the patient's serum showed a strikingly linear but not granular IgG pattern along the epithelial basement membranes of monkey esophagus and kidney. Although type XVII collagen was recently identified in the glomerulus, the patient's serum did not produce a 180-kDa band on immunoblot of kidney tissue and still stained glomeruli of BP180 knockout mice by indirect immunofluorescence. The patient was treated with prednisone and azathioprine, which resulted in complete remission of skin and kidney manifestations. Although bullous pemphigoid has been reported previously in association with anti-glomerular basement membrane disease or membranous nephropathy, this case demonstrates both elements in 1 patient. This concurrence and the linear pattern on indirect immunofluorescence support the possibility of cross-reactive or parallel autoantibodies to basement membranes with a secondary membranous component. (C) 2016 by the National Kidney Foundation, Inc. |
| Databáze: | OpenAIRE |
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