Abstract
A rare and a disease of unknown etiology, Churg-Strauss syndrome (CSS) is a granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia, which is initially described by Churg and Strauss in 1951. Because of its clinical and pathological features that overlap with those of the other anti-neutrophil antibody (ANCA)-associated systemic vasculitides (AASVs) and now the disease is classified as AASVs. The ANCA status may dictate the clinical phenotype. ANCA-positive patients are significantly more likely to have disease manifestations associated with small-vessel vasculitis, including oecrotising glomemlonephritis, mononeuritis and purpura, whereas ANCA-negative cases predominantly likely to have cardiac and lung involvement. The objective of this case report is to point out the possibility of vasculitic rash in ANCA-negative CSS in a 35-year-old man and the disease rarely occurs in Bangladeshi population. We analyze the history, clinical examinations and relevant investigations related to the patient to establish the diagnosis in our department. The clinical scenario and biopsy help us to attain the diagnosis. But due to unavailability of patients' cohort we have limitations of comparison of ANCA status in Bangladeshi populations. Though ANCA-positive and ANCA-negative CSS differ phenotypically, primary therapy for both the conditions is systemic glucocorticoids. Additional immunosuppressive agents like cyclophosphamide, mycophenolate mofetil, azathioprine, rituxin1ab are occasionally added in patients with more advanced or refractory disease.
Highlights
Churg-Strauss syndrome (CSS) is a rare small and medium vessel vasculitis characterrzed by eosinophilic infiltration of organs with necrotizing vasculitis and interstitial and perivascular granulomas '
American College of Rheumatology (ACR) has proposed six criteria for CSS-four being necessary for CSS to be diagnosed with 85% sensitivity and 99.7% specificity.[6]
Syed Jamil Abdal et al studies continue to show that CSS is the rarest of the necrotizing small-vessel vasculitides.ll The mean age at diagnosis of CSS is 40 years which was compatible in our casel[2]
Summary
Churg-Strauss syndrome (CSS) is a rare small and medium vessel vasculitis characterrzed by eosinophilic infiltration of organs with necrotizing vasculitis and interstitial and perivascular granulomas '. A 35-year-old, man, with history of bronchial asthma for last 3 years, presented to us with the complaints of fever for one and half months, appe arafice of rashes over both t78. ANCA-neeative Churg-Straus Swdrome: A Case Report for one and half months, appe arance of rashes over both lower limbs along with unable to move his right hand for last 7 days due to weakness. He developed low-grade fever, malaise, lassitude, loss of appetite and generahzedbody-ache.
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