Think Zebras: Late Stage of Churg–Strauss Syndrome

1Queen's Medical Center, Honolulu, HI

Meeting: Hospital Medicine 2011, May 10-13, Dallas, Texas.

Abstract number: 383

Case Presentation:

A 61‐year‐old woman presented to the emergency department with a painful, nonpruritic rash over the palms of the hands, elbows, face, and behind the ears for 1 week. Associated symptoms included weakness and shooting pain in the left lower extremity. She noted progressive worsening of the presenting symptoms after completion of prednisone taper for recent asthma exacerbation. The patient was diagnosed with asthma and allergic rhinitis less than 1 year ago. She reported frequent asthma exacerbations requiring multiple courses of prednisone. Skin examination revealed tender, violaceous, nonblanching nodules on the palms of the hands, face, scalp, elbows, and posterior auricular. Neurological exam was significant for 1/5 strength in the left ankle dorsiflexion. Initial workup revealed leukocytosis of 28,700/μL with 54% eosinophils. Given the constellation of symptoms of allergic rhinitis, asthma, prominent peripheral blood eosinophilia, and vas‐culitic‐appearing rash, a diagnosis of Churg–Strauss syndrome (CSS) was suspected. Punch biopsy of the left hand showed acute vasculitis with prominent eosinophilic infiltrate without granulomas. Marked eosinophilic infiltrate favored Churg–Strauss syndrome. The patient was started on intravenous steroids and showed marked symptomatic improvement over 48 hours. Leukocytosis decreased to 16,000/μL with 3% eosinophils. Seven days after discharge on oral prednisone, the patient's rash continued to improve, and eosinophilia remained stable at 3%. Left lower‐extremity weakness and paresthesias persisted.

Discussion:

CSS is characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. Most commonly affected organs include lung and skin; cardiovascular, gastrointestinal, renal, and central nervous systems can also be involved. The etiology is unknown. CSS occurs in the following stages. The prodromal phase is characterized by allergic rhinitis, asthma, and atopy. The eosinophilic phase includes peripheral blood eosinophilia and eosinophilic organ infiltration. The vasculitic phase is a systemic small‐ and medium‐vessel vasculitis with skin involvement and vasculitic neuropathy being common features. Diagnosis of CSS remains a challenge because it a rare condition (2.4–6.8 cases per 1,000,000 patient/year), manifestations may occur in isolation and be separated by decades, and there are no laboratory tests specific for this condition. The American College of Rheumatology diagnostic criteria appear to be the most comprehensive. The primary therapy for CSS is systemic glucocorticoids. Refractory CSS may respond to cyclophosphamide, azathioprine, intravenous immune globulin, and interferon‐alpha.

Conclusions:

CSS is a rare condition that presents a diagnostic challenge. In the inpatient setting, patients with CSS are likely to present in the advanced stages and require rapid diagnosis and treatment. Awareness of this condition and clinical suspicion are critical in the appropriate management of patients with CSS.

Disclosures:

M. Roytman ‐ Queen's Medical Center, employment.

To cite this abstract:

Roytman M. Think Zebras: Late Stage of Churg–Strauss Syndrome. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 383. https://www.shmabstracts.com/abstract/think-zebras-late-stage-of-churgstrauss-syndrome/. Accessed December 10, 2018.

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