[56-year-old patient with leg paresis, pulmonary infiltrates, and eosinophilia--Case 08/2009].
METHODS
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.


METHODS
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).


METHODS
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.


CONCLUSIONS
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.
Henes
Jörg
J
Medizinische Universitätsklinik Tübingen, Abteilung für Onkologie, Hämatologie, Rheumatologie, Immunologie, Pulmonologie. joerg.henes@med.uni-tuebingen.de
Horger
Marius
M
Kanz
Lothar
L
Kötter
Ina
I
D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D000974:Antibodies, Antinuclear / Q000097:blood; D001379:Azathioprine / Q000627:therapeutic use; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000628:therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004417:Dyspnea; D004802:Eosinophilia / Q000209:etiology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008168:Lung / Q000000981:diagnostic imaging* / Q000473:pathology; D008875:Middle Aged; D010291:Paresis / Q000209:etiology; D011115:Polyneuropathies; D011239:Prednisolone / Q000627:therapeutic use; D012121:Respiration, Artificial; D014057:Tomography, X-Ray Computed
D019268:Antibodies, Antineutrophil Cytoplasmic; D000974:Antibodies, Antinuclear; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone; D001379:Azathioprine