CUTANEOUS LEUKOCYTOCLASTIC VASCULITIS AND MEMBRANOPROLIFERATIVE - PowerPoint PPT Presentation

CLINICAL-PATHOLOGICAL CONFERENCE CUTANEOUS LEUKOCYTOCLASTIC VASCULITIS AND MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS WITH MONOCLONAL IGM GAMMOPATHY . Antonello Pani Divisione di Nefrologia, Dialisi e Trapianto AO Brotzu, Cagliari (CA), IT

� Hematuria is fairly common and can suggest severe and benign diseases from the upper to the lower urinary tract. � The nephrologist have to assess: � The glomerular or different nature (acanthocytes in urine sediment) of hematuria. � Is it really «isolated»?

Leukocytoclastic Cutaneous Vasculitis � Palpable purpura characterized by a neutrophil infiltrate of the arterial or venule vessel walls. Palpable Palpable Purpura Purpura Chapel Hill consensus conference in Systemic 1994 Isolated Disease Immunoglobulins Few or no-immune Leukocyt in the vascular mixed deposits walls cryoglobulinemia oclastic (type II or type III) Purpura with IgG and C 3 deposits Microscopic Pauci-immune Microscopic Henoch- polyangiitis nature Polyarteritis Schonlein purpura (IgA deposits)

Others patterns of Renal-Cutaneous Syndrome, a literature review. � Paraneoplastic syndromes of Renal Cancers � Vasculitides � Polyangiitis Microscopic � Wegener Granulomatosis � Autoimmune diseases � Sjogren Syndrome � Systemic Lupus Erythematosus � Chronic inflammatory bowel diseases � Infectious diseases � HCV � Influenza Virus � Parvovirus B19 � Pseudomonas Aeruginosa � Drugs � Naproxen, Rifampin, Ciprofloxacin, Warfarin, Dapsone, Allopurinol and others

Patterns of Renal Injury related to Plasmacells Dyscrasias � Amyloidosis AL and AH � Monoclonal Immunoglobulin Deposition Diseases (MIDD) � Light Chain Deposition Disease (LCDD) � Heavy Chain Deposition Disease (HCDD) � Light & Heavy Deposition Disease (LHCDD) � Monoclonal Cryoglobulinemia � Proliferative glomerulonephritis due to monoclonal immunoglobulin deposition (ICMDD) (Waldenstrom Macroglobulinemia, IgM Myeloma, IgM secreting Non and Hodgkin B cell Lymphoma) � Fibrillary Glomerulonephritis and Immunotactoid Glomerulopathy � Lymphomatous Infiltrate � Crescentic Glomerulonephritis Audard V, Georges B, Vanhille P, Toly C, Deroure B, Fakhouri F, Cuvelier R, � Cast Nephropathy Belenfant X, Surin B, Aucouturier P, Mougenot B, Ronco P. Renal lesions associated with IgM-secreting monoclonal proliferations: � Fanconi Syndrome revisiting the disease spectrum. Clin J Am Soc Nephrol. 2008 Sep;3(5):1339-49. Epub 2008 Jul 16.

Nothing of these etiologies have been found in our patient. � HCV, HBV, HIV or other viral/bacterial infections weren � t in act � C4 was on limits and Cryo and Hypocryo Precipitation Tests were negatives � No signs or immunological lab tests positive for an active autoimmune/connective disorder in every determination. � No therapies were in act

The Renal Biopsy � The sudden worsening of the renal function with the characteristics of RPGN , the persistent microscopic hematuria, the mild C3 consumption and the mild proteinuria made the renal biopsy necessary. � MGUS IgM-K o Waldenstrom Macro- globulinemia? In all determinations of Bone Marrow aspirates there weren � t signs of active pathology.