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A 47-year-old male presents with declining renal function characterized
by oliguria, elevated blood urea nitrogen and creatinine, and hematuria .
He also complains of nasal congestion and epistaxis. Review of systems is
notable for occasional cough and hemoptysis. Examination shows mucosal
ulceration and nasal septal perforation, but no polyps. Which of the
following serum markers would likely be present in this case?
A. Anti-centromere antibody
B. Anti-Ro
C. Anti-SS-B
D. c-ANCA (cytoplasmic antinuclear cytoplasmic antibody)
E. Decreased erythrocyte sedimentation rate (ESR)

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Explanation:
The correct answer is D. This patient has Wegener's granulomatosis, which is characterized by renal involvement, severe upper respiratory tract symptoms, and pulmonary involvement. Other organ systems may also be involved. The renal syndrome is a crescentic rapidly progressive glomerulonephritis leading to renal failure. The upper respiratory tract findings include sinus pain and drainage, and purulent or bloody nasal discharge with or without nasal ulcerations. Nasal septal perforation may follow. Pulmonary involvement may be clinically silent with only infiltrates present on x-ray, or it may present as cough and hemoptysis. c-ANCA is a marker for Wegener's granulomatosis, present in a high percentage of patients.
Anti-centromere antibody (choice A) is associated with approximately 90% of cases with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal motility syndrome, sclerodactyly, and telangiectasia) which is also called limited scleroderma.
Anti-Ro (choice B) is also called anti-SS-A and is associated with Sjِgren's syndrome (70-95%).
Anti-SS-B (choice C) is associated with Sjِgren's syndrome (60-90%).
Decreased ESR (choice E) is not a marker of Wegener's. Instead, a markedly elevated ESR is seen. Additionally, mild anemia, thrombocytosis, leukocytosis, mild hypergammaglobulinemia (IgA), and mildly elevated rheumatoid factor are seen in this disorder.

Ans D
upper RTI + Lower RTI + renal --> wegner's granuloamtosis ---> cANCA

DDD