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#15

ANSWER B.

This patient has Granulomatosis with Polyangiitis (GPA) (formerly referred to as Wegener’s granulomatosis) as demonstrated by hemoptysis, hematuria, and renal insufficiency. GPA is a multisystem vasculitis with necrotizing granulomas that involve the pulmonary and renal systems. The most common presenting symptoms are hemoptysis and hematuria. The upper and lower airways, as well as the ears, may be involved. Non-specific symptoms such as fatigue and joint pain are often present. Glomerulonephritis with renal insufficiency can occur. Skin findings include cutaneous nodules and palpable purpura. Cardiac involvement is rare but potentially fatal. Treatment involves stabilizing the patient and corticosteroids as well as immunosuppressant agents such as cyclophosphamide in conjunction with a specialist.  
 
Churg Strauss syndrome (A) is a multisystem vasculitis that presents with bronchospasm (progressing to possible airway obstruction), as well as sinusitis. Peripheral eosinophillia is often present. Cardiac and gastrointestinal symptoms may also occur; however, hemoptysis and hematuria are less common in Churg Strauss than in granulomatosis with polyangiitis. Polyarteritis nodosa (C) is also a multisystem vasculitis. While its presentation generally involves non-specific systemic complaints, organ-specific complications such as renal failure, gastrointestinal bleeding, cardiac ischemia, and stroke may also be seen. Pulmonary involvement is relatively uncommon. Systemic sclerosis (D) is characterized by excessive collagen deposition with vascular and endothelial dysfunction. Patients often present with thickened skin and ischemic digits along with gastrointestinal symptoms, possibly due to stricture formation. The renal and pulmonary systems can also be involved but the patient in this scenario does not have any other symptoms consistent with systemic sclerosis.

Ref
Morabito GC, Tartaglino: Emergencies in Systemic Rheumatic Diseases, in Tintinalli JE, Stapczynski JS, Ma OJ, et al (eds): Tintinalli’s Emergency Medicine, ed 7. New York, The McGraw-Hill Companies Inc., 2011, (Ch) 279:p 1911-1920

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Comments   

# Mini Board Review: March 2013 EditionDiane Gilliland 2013-03-07 20:37
Thank you for putting together the questions for the mini board review. I find them very helpful!
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# Mini Board reviewDr Zahn 2013-03-10 09:37
Good review....but alot of medical info, very specific...not known when you are out for years in practice.
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# MDSam Morale 2013-03-11 17:06
Thanks for your effort. Nice to review uncommon diseases to keep us up to speed. It is amazing how many diseases, pathogens etc... have had their names changed : Granulomatosis with Polyangiitis (GPA) (formerly referred to as Wegener’s granulomatosis) .
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# Robert Ditrolio 2013-03-12 17:10
Excellent review questions. Please keep them coming in future issues!
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