Henes J, Horger M, Kanz L, Kötter I
[56-year-old patient with leg paresis, pulmonary infiltrates, and eosinophilia--Case 08/2009] [Case Reports, English Abstract, Journal Article]
Dtsch Med Wochenschr 2009 Oct; 134(44):2228.
HISTORY AND ADMISSION
FINDINGS: A 56-year-old woman presented with progressive dyspnea and polyneuropathia. Medical history revealed a bronchial asthma and hypertension. She was in a reduced general condition and had to be admitted to the intensive care unit for mechanical ventilation the same day.
INVESTIGATIONS: The computed tomography of the chest revealed distinct pulmonary infiltrates. Laboratory findings showed significantly elevated inflammatory markers as well as an eosinophilia in the differential blood count and the bronchial lavage. Diagnostics for infections were all negative as were antinuclear and anti-neutrophil cytoplasmatic antibodies (ANCAs).
DIAGNOSIS, TREATMENT AND COURSE: An ANCA negative Churg Strauss Syndrome (CSS) was diagnosed in accordance with extravascular and blood eosinophilia, pulmonary infiltrates and the neurological symptoms. Aggressive therapy with high dose glucocorticosteroids and cyclophosphamide (CYC) pulses was initiated. The patient improved rapidly and was extubated 6 days after the initiation of treatment. After 6 cycles of CYC with 750 mg/m2 and a maintenance treatment with azathioprine and 5mg prednisolone the patient is still in complete remission 2,5 years after the diagnosis.
CONCLUSION: The CSS belongs to the ANCA-associated vasculitides. The patient described here presented with all the characteristic organ involvements of CSS and a fulminate worsening. The fast diagnosis and aggressive therapy which was started only hours after admission to the intensive care unit induced a long lasting remission.
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