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What is 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 12000/mm3, red cell casts in the urine and serum creatinine 3mg/dL. What is the most probable diagnosis? and explain in detail?
Answer is C (Wegener's 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 Wegener's granulomatosis. Wegener's 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. Wegener's granulomatosis: Review Wegener's 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 class)Q * Hypocomplementemia is not seen despite presence of circulating immune complexes" Treatment of choice is: cyclophosphamide Q