Case Report: A 72-year-old woman with hemoptysis and bronchial asthma
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72-yo female patient presented with cough, arthralgia, numbness, loss of sensitivity, and muscle pain of lower limbs. Year later she was admitted in Rheumatology department with fever, hemoptysis, electroneuronography data for multiplex mononeuritis.
Patient’s lab tests showed anemia, high ESR and CRP, elevated levels of eosinophils in peripheral blood, positive p-ANCA and anti-MPO. The patient has a history of bronchial asthma. Her plain X-ray revealed diffuse bilateral infiltrations due to alveolar hemorrhage. In order to verify that infiltrations are due to alveolar hemorrhage, bronchoalveolar lavage was done.
The initiated treatment was pulse doses of methylprednisolone (1000 mg once monthly) and cyclophosphamide (1000 mg once monthly) for six months, high doses of glucocorticoids in between. After the induction therapy, glucocorticoids were tapered to low dose and azathioprine 2.5 mg/kg bid were introduced.
Diagnosis
Diagnostic markers include eosinophil granulocytes and granulomas in affected tissue and antineutrophil cytoplasmic antibodies against neutrophil granulocytes. The American College of Rheumatology 1990 criteria for diagnosis of Churg–Strauss syndrome lists these criteria:
- Asthma
- Eosinophils greater than 10% of a differential white blood cell count
- Presence of mononeuropathy or polyneuropathy
- Unfixed pulmonary infiltrates
- Presence of paranasal sinus abnormalities
- Histological evidence of extravascular eosinophils
For classification purposes, a patient shall be said to have Churg–Strauss syndrome (CSS) if at least four of these six criteria are positive. The presence of any four or more of the six criteria yields a sensitivity of 85% and a specificity of 99.7%. [1]
1. Masi AT, Hunder GG, Lie JT, et al. (August 1990). "The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis)". Arthritis Rheum. 33 (8): 1094–100. doi:10.1002/art.1780330806. PMID 2202307.
Tags: churg-strauss, eosinophilic granulomatosis, eosinophilic granulomatosis with polyangiitis