<prompt> A 20 year old woman presents with bilateral conductive deafness, palpable purpura on the legs and hemoptysis. Radiograph of the chest shows a thin-walled cavity in left lower zone. Investigations reveal total leukocyte count 12000mm3, red cell casts in the urine and serum creatinine 3mgdL. What is the most probable diagnosis?Henoch-Schonlein purpuraPolyaeritis nodosaWegeners granulomatosisDisseminated tuberculosis  <response>: Answer is C Wegeners granulomatosis The patient in question has been depicted to have one manifestation each of the classical triad as mentioned below along with skin lesion in the _Pm of palpable purpura and hence the answer is clearly Wegeners granulomatosis. Wegeners granulomatosis in its classical form presents as characteristic triad of ? - Upper respiratory infection ENT manifestations: includes otitis media conductive deafness - Lower respiratory tract infection Lung: includes haemoptysis and radiological cavity. - Renal involvement: includes red cell casts and raised serum creatinine. Skin lesions are seen in 46 of patients and may take the form of palpable purpura. Wegeners granulomatosis: Review Wegeners granulomatosis is a distinct clinicopathological entity characterized ? Clinically Triad of Upper respiratory tract manifestation ENT Lower respiratory tractdeg manifestations Lung Renal manifestations - Otitis media conductive deafness - Paranasal sinus pain drainage - Septal perforationQ Other manifestations - Cough, haemoptysis, dyspneaQ - Multiple bilateral cavitatory nodular infiltrates - Rapidly progressive rena failureQ - Crescentric glomerulonephritisQ FeverQ, skin manifestationQ in the form of papules, vesicles, palpable purpura etc eye manifestationsQ, joint manifestationsQ cardiac manifestationsdeg and nervous system manifestationsQ. Pathologically by Necrotizing vasculitis of smalls aeries and veins Intravascular and extravascular granuloma formation Q Granulomas contain multiple well defined multinucleated giant cells Bronchoalveolar lavage fluid contains high percentage of neutrophils compared to other granulomatous diseases which contain increased number of Lymphocytes Q c-ANCA is the predominant ANCA in the disease Characteristic laboratory findings include: I . Positive c-ANCA0 2. Elevated ESR mild elevation 3. RFdeg often elevated 4. Hypergammaglobunemia paicularly IgA classQ Hypocomplementemia is not seen despite presence of circulating immune complexes Treatment of choice is: cyclophosphamide Q 