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A 28-year-old female nurse at a skilled nursing facility with a medical history remarkable only for depression presented to an outside emergency department (ED) in October 2007 with maxillary sinus tenderness, tinnitus, and otalgia of 3 weeks' duration. She had been married for 5 years and had no history of foreign travel. Her family history was only remarkable for a maternal uncle with sarcoidosis. At the time of her evaluation, a physician noted that the patient had cervical lymphadenopathy and made a diagnosis of acute sinusitis and otitis media. The patient was prescribed amoxicillin-clavulanate and discharged home. After 1 week of antibiotic therapy, all of the symptoms had improved.
In the following month, she developed intermittent flu-like symptoms of cough, sore throat, fatigue, and night sweats. During the following 2 months, she unintentionally lost more than 30 lbs (13.6 kg). For these reasons, she was admitted to her local hospital.
On admission, she was afebrile with marked cervical, supraclavicular, and axillary lymphadenopathy. Laboratory testing disclosed marked peripheral eosinophilia of 2300/μL, which was 15% of the white blood cell differential. Computed tomography (CT) of the chest showed normal lung parenchyma but demonstrated substantial bilateral hilar adenopathy. A cervical lymph node biopsy revealed reactive hyperplasia but was negative for malignancy and granulomas. A week after hospitalization and supportive therapy, the patient was discharged without a diagnosis; her fever and sore throat spontaneously resolved.
Two months after her hospitalization, the patient began experiencing a painful, asymmetrical, multifocal weakness that was worse in the lower extremities and more prominent on the right. According to the patient, her weakness was persistent and neither worsened with exercise nor improved with rest. Her initial neurologic examination was remarkable for a right foot drop and considerable right lower extremity weakness, but after 2 weeks she developed bilateral foot drop and symmetrical bilateral lower extremities weakness.
In the next 2 months, she experienced symmetrical weakness in her lower extremities and became wheelchair-bound. She was readmitted to the hospital. Findings on CT of the head were negative. Electromyography (EMG) and nerve conduction studies (NCSs) showed reductions in both conduction velocity and the amplitude of sensory and motor action potentials. Additional laboratory tests showed a fasting serum glucose level of 90 mg/dL (reference ranges provided parenthetically) (70-100 mg/dL) and were negative for Epstein-Barr virus IgM and IgG; cytomegalovirus IgG and IgM; extractable nuclear antigen panel (Sjögren syndrome antigen A, Sjögren syndrome antigen B, scleroderma-70 antigen, antihistidyl—transfer RNA synthetase, Smith antibodies, ribonucleoprotein); Lyme IgM and IgG; human immunodeficiency virus; hepatitis A, B, and C; perinuclear antineutrophil cytoplasmic antibody (ANCA); and cytoplasmic ANCA. However, she continued to have elevated levels of peripheral eosinophils (15%) and a mildly elevated antinuclear antibody level (5.1 U [≥3 U is positive]). A muscle biopsy was unremarkable for myositis. A lumbar puncture with cerebrospinal fluid (CSF) studies yielded the following results: erythrocyte count, 1/mm3 (0/mm3); leukocyte count, 1/mm3 (0-3/mm3); glucose, 48 mg/dL (40-85 mg/dL); total protein, 95 mg/dL (15-50 mg/dL); and a normal level of angiotensin-converting enzyme.
Churg-Strauss syndrome is a small-vessel vasculitic disease that is characterized by sinusitis, asthma, marked peripheral eosinophilia, and peripheral neuropathy. The mean age at diagnosis of CSS is 50 years, but nonspecific constitutional symptoms of malaise, fevers, and weight loss can appear in those aged 30 to 40 years. Adult-onset asthma is the most common feature of CSS and is estimated to occur in 95% of patients with CSS.9 Asthma can precede other symptoms by up to 10 years.
The American College of Rheumatology established 6 criteria to aid clinicians in making the diagnosis of CSS.5 Meeting 4 of these 6 criteria would yield a sensitivity of 85.0% and a specificity of 99.7% for the diagnosis of CSS. Interestingly, the testing of ANCA is not helpful in the diagnosis of CSS because less than 40% of patients with CSS are ANCA-positive.10
Surgical biopsy of affected lung tissue is considered the criterion standard in the diagnosis of CSS, whereas transbronchial lung biopsy is rarely useful. Typical histological features of CSS include extensive eosinophilic infiltrates, vasculitis, and granulomatous inflammation. Lanham et al11 make the point that CSS is typically underdiagnosed because of the emphasis on histological demonstration of eosinophilic infiltrates, extravascular granulomas, and vasculitis, the last of which is found only in a minority of cases. Lanham et al suggest that CSS can be identified solely on clinical findings.
The mainstay therapy for CSS is high-dose corticosteroids tapered slowly over the course of 12 weeks or until symptoms have resolved. Early withdrawal of corticosteroids can cause CSS relapse. Patients' symptoms, serum eosinophil level, and erythrocyte sedimentation rate should be routinely monitored for responsiveness to therapy. For patients who are nonresponsive to corticosteroids, other novel treatment options should be considered, including cyclosporine, intravenous immunoglobulin, and anti-IgE therapy.6-8
Correct answers: 1. c, 2. d, 3. e, 4. b, 5. a