Anti-neutrophil cytoplasmicantibodies (ANCA)is seen in:
Correct Answer: Wegener's Granulomatosis
Description: Ans. a. Wegener's Granulomatosis (Ref: Harrison 19/e p2182, 18/e p2786)Anti-neutrophil cytoplasmic antibodies (ANCA) are seen in Wegener's Granulomatosis." Wegener's Granulomatosis: c-ANCAs are present in the serum in up to 95 % of the patients with active generalized disease, and this appears to be a good marker for disease activity."ANCA (Antineutrophilic cytoplasmic antibodies)* ANCA are antibodies directed against certain proteins in cytoplasmic granules of neutrophils and monocytes.* There are two major categories:C-ANCA(Cytoplasmic protein 3 is the target antigen)P-ANCA(Perinuclear myeloperoxidase is the major target antigen)* Wegner's GranulomatosisQ (90-95%)* Microscopic PAN (microscopic poiyangitis)Q* Churg-Strauss syndromeQ* Crescentic GlomerulonephritisQ* Good Pasteur's syndromeQHistory of chronic sinusitis, nasopharyngeal ulcers, cavitatory lung nodules and renal failure are suggestive of Wegener's Granulomatosis. Wegener's Granulomatosis is c-ANCA positive small vessel vasculitisQ c-ANCA levels are required for diagnosis."The histopathologic hallmarks of granulomatosis with polyangiitis (Wegener's) are necrotizing vasculitis of small arteries and veins together with granuloma formation, which may he either intravascular or extravascular. Lung involvement typically appears as multiple, bilateral, nodular cavitary infiltrates, which on biopsy almost invariably reveal the typical necrotizing granulomatous vasculitis. Upper airway lesions, particularly those in the sinuses and nasopharynx, typically reveal inflammation, necrosis, and granuloma formation, with or without vasculitis. "-- Harrison 19/e p2182"The specificity of a positive antiproteinase-3 ANCA for granulomatosis with polyangiitis (Wegener's) is very high, especially if active glomerulonephritis is present. However, the presence of ANCA should he adjunctive and, with rare exceptions, should not substitute for a tissue diagnosis. "-- Harrison 19/e p2184ANCA (Antineutrophilic cytoplasmic antibodies)ANCA are antibodies directed against certain proteins in cytoplasmic granules of neutrophils & monocytes.There are two major categories:Wegener's GranulomatosisCharacterized clinically by granulomatous vasculitis of the upper & lower respiratory tracts together with glomerulonephritis & variable degrees of disseminated vasculitis involving both small arteries & veins.c-ANCA positive small vessel vasculitisQPathology:Necrotizing vasculitisQ of small arteries & veinsQIntravascular & extravascular granuloma formationQGranulomas contain multiple well defined multinucleated giant cellsQBronchoalveolar lavage fluid contains high percentage of neutrophils compared to other granulomatous diseases which contains increased number of LymphocytesClinical Features:Fever, skin manifestation in the form of papules, vesicles, palpable purpura etc, eye manifestations, joint manifestations, cardiac manifestations & nervous system manifestationCharacteristic Triad of Wegener's GranulomatosisVasculitis of upper respiratory tractQ (ENT)Vasculitis of Lower respiratory tractQ (Lung)Vasculitis of Kidney* MC (95%)* Otitis mediaQ (conductive deafness)* Paranasal sinus pain & drainage* Septal perforationQ* Sinusitis, subglottic stenosisQ* Strawberry gum & gum ulcersQ* Mucosal ulcerations of nasopharynx* 2nd MC (85-90%)* Cough, hemoptysis, dyspneaQ* Multiple bilateral cavitatory nodular infiltrates & cavitatory lesions in the lungQ* Seen in 80% patients* Rapidly progressive renal failureQ* Crescentic glomerulonephritisQ* Present with hematuria, proteinuria & RBC cast in urineQLess common Vasculitis associated with Wegener's GranulomatosisOrgan involvedManifestationsSkin (46%)* Palpable purpura, subcutaneous nodulesQ, ulcers, vesicobullous lesions, splinter hemorrhages, digital ischemia & gangreneJoints* Arthralgia & migratory arthritisQEyes (52%)* Orbital pseudotumors behind the eye leading to proptosis & visual loss* Scleritis, keratitis, conjunctivitis & uveitisPeripheral nerve* Mononeuritis multiplexQCharacteristic laboratory findings of Wegener's Granulomatosis* Positive c-ANCAQ* Elevated ESR, RFQ(mild elevation)* Anemia, leukocytosis & thrombocytosisQ* Hypergammaglobulinemia (particularly IgA class)Q* Hypocomplementemia is not seen despite presence of circulating immune complexesQTreatment:Treatment of choice: CyclophosphamideQCyclophosphamide is given in doses of 2 mg/kg per day together with glucocorticoidsQ
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