[Acute coronary syndrom as a cardiac manifestation of granulomatosis with polyangiitis].

HISTORY AND ADMISSION FINDINGS: A 49-year-old patient was admitted to our ward because of a troponin elevation (non ST-elevation myocardial infarction) following a rhinoscopy in an external hospital. The patient complained of typical angina, chronic rhinitis and epistaxis. Analysis of the nasal biopsy had shown the histological finding of granulomatosis with polyangiitis (Wegener's granulomatosis).

INVESTIGATION: The consecutively performed catheterization showed a coronary one-vessel disease without significant stenosis. Echocardiography showed diastolic dysfunction as well as hemodynamically not significant pericardial effusion. The MRI scan of the heart revealed multiple myocardial scars located ventricular apical and septal. Extended bilateral pulmonary opacities in the thoracic CT scan, microhematuria, leukocyturia and proteinuria indicated multi-organ involvement of the small vessel disease.

TREATMENT AND COURSE: The patient's condition improved quickly in response to steroids and cyclophosphamide, followed by attenuation of clinical symptoms and normalizing blood and renal parameters.

CONCLUSION

The prognosis of granulomatosis with polyangiitis is mainly limited by renal and pulmonal involvement. Cardiac involvement is commonly rare and associated with clinical courses refractory to immunosuppressive therapy. Generally, all cardiac structures can be affected, thereby impending serious cardiac events. Normally, granulomatosis with polyangiitis responds quickly to immunosuppressive therapy, associated with a rather good prognosis without higher mortality.