A 25 years old female develops serous otitis media of left ear with cough and occasional hemoptysis and hematuria and epistaxis for one and half months her hemoglobin is 7 gm. B.P is > 170/100, proteinuria +++, RA – ve and ANCA – ve, the likely cause is?
Correct Answer: Wegener's granulomatosis
Description: Answer is A>D (Wegener's granulumatosis > Goodpasture's Syndrome); The patient in question is presenting with characteristic clinical triad of Wegener's granulomatosis, namely, Vasculitis of upper respiratory tract (serous otitis media, epistaxis), Vasculitis of lower respiratory tract (cough and hemoptysis) and vasculitis of the kidney (proteinuria)Although positive ANCA assays are often instrumental in suggesting the diagnosis of WG, Negative ANCA assays do not preclude the diagnosis, 10% of patients with disseminated WG and upto 30% of patients with limited WG may be negative for ANCAThe clinical picture is classical of Wegner's Granulomatosis. However, the absence of ANCA confuses the diagnosis. Neveheless the presence or absence of ANCA should be adjunctive and in the presence of a classical clinical picture of WG, tissue diagnosis (biopsy) should be performed to confirm the diagnosis.Definitive diagnosis of WG is established by tissue biopsy and not by presence or absence of ANCAPulmonary + Renal Syndrome Pulmonary (Hemoptysis) + Renal (Proteinuria) manifestationsWegener's granulomatosisClassical Clinical presentation is one of a young Male/Female (male: female=1:1) presenting with upper respiratory tract features (Otitis media, Epistaxis) along with hemoptysis and acute glomerulonephritis+ANCA is usually positive and suggests the diagnosisNegative ANCA does not preclude the diagnosis. In the presence of classical clinical picture a provisional diagnosis of WG should be made on clinical ground and confirmed/ excluded by tissue biopsyThe patient in question is presenting with classical clinical triad of Wegener's granulomatosis. Although ANCA is negative a negative ANCA does not preclude the diagnosis of WG. Based on the strong clinical suspicion, WG should the single best initial provisional diagnosis and tissue biopsy should be performed.Goodpasture's syndrome Classical clinical presentation is one of a young male smoker (male female=3-4:1) presenting with hemoptysis and signs of acute glomerulonephritis Although disease may be preceeded by an upper respiratory tract infection, upper respiratory tract involvement features like otitis media and epistaxis are not characterstic of good pasture's syndrome ANCA is usually negative Diagnosis is made by detecting serum levels of Anti GBM antibodies The patient in question is not presenting with classical clinical features of good pasture's syndrome and serum levels of Anti GBM antibodies have not been provided in the question. These make a diagnosis of Good pasture's syndrome less likely.
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