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.

Chapel Hill consensus conference in 1994

Palpable Purpura Palpable Purpura Isolated Systemic Disease

Immunoglobulins in the vascular walls Few or no-immune deposits

Leukocyt

• clastic

Purpura

Microscopic Polyarteritis Microscopic polyangiitis Pauci-immune nature

Henoch- Schonlein purpura (IgA deposits) mixed cryoglobulinemia (type II or type III) with IgG and C3 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

• thers

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 Cast Nephropathy Fanconi Syndrome

Audard V, Georges B, Vanhille P, Toly C, Deroure B, Fakhouri F, Cuvelier R, Belenfant X, Surin B, Aucouturier P, Mougenot B, Ronco P. Renal lesions associated with IgM-secreting monoclonal proliferations: 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

werent 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

• Waldenstrom

Macro- globulinemia? In all determinations of Bone Marrow aspirates there werent signs of active pathology.