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[Eosinophilic Granulomatosis with Polyangiitis with Pulmonary and Cardiac Involvement].
- Source :
-
Pneumologie (Stuttgart, Germany) [Pneumologie] 2021 Jan; Vol. 75 (1), pp. 33-38. Date of Electronic Publication: 2020 Jul 24. - Publication Year :
- 2021
-
Abstract
- A 62-year-old patient with bronchial asthma and chronic rhinosinusitis underwent inguinal hernia surgery. After the operation, sudden circulatory arrest occurred, requiring cardiopulmonary resuscitation. Coronary angiography revealed a 99 % proximal stenosis of right coronary artery (RCA) with unsuspicious and smooth coronary vessel walls. In the further course, several similar events occurred, but without pathological findings in the coronary angiography. Initially, echocardiography showed slightly reduced left ventricular ejection fraction of 45 %. Chest radiography revealed bilateral pulmonary infiltrates, and white blood cell count showed severe eosinophilia (37 %). Serological antibody testing including ANA, ENA and c-/p-ANCA was negative. Myeloproliferative pathologies were excluded by bone marrow puncture. The patient suffered from emerging dyspnea, weakness, and ongoing weight loss. A methylprednisolone pulse of 250 mg/d for 3 days remained without significant effect, so that the patient was eventually referred to our university hospital due to ongoing clinical deterioration. On admission, the patient suffered from weakness, progressive muscular atrophy, and dyspnea on exertion. Physical examination revealed a right-sided peroneal paralysis. Bronchial lavage detected severe eosinophil alveolitis (37 %), and laboratory findings showed elevated cardiac enzymes and NT-proBNP (Troponin-T > 700 ng/l, NT-proBNP > 10.000 ng/l). Echocardiography revealed a dramatic deterioration of cardiac function (LVEF 16 %). Interdisciplinary discussion between pulmonologists and cardiologists lead to the diagnosis of ANCA-negative eosinophilic granulomatosis with polyangiitis (EGPA) with pulmonary and cardiac involvement. Initiation of immunosuppressive therapy with methylprednisolone 1000 mg/d for 3 days followed by cyclophosphamide therapy (6 pulses, administered every 4 weeks) led to substantial symptomatic improvement, complete regression of pulmonary infiltrates and marked recovery of cardiac function (LVEF 47 %). CONCLUSION: Serological detection of elevated ANCAs is not necessary for diagnosis of EGPA. Only 30 - 70 % of patients are positive for these, particularly if neurological and/or renal rather than cardiac and/or pulmonary involvement is present. This may be a pitfall in establishing the correct diagnosis. Induction therapy with cyclophosphamide is the preferred treatment for steroid-refractory EGPA with life-threatening organ involvement.<br />Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht.<br /> (Thieme. All rights reserved.)
- Subjects :
- Antibodies, Antineutrophil Cytoplasmic therapeutic use
Churg-Strauss Syndrome drug therapy
Coronary Angiography
Coronary Stenosis
Cyclophosphamide therapeutic use
Dyspnea etiology
Echocardiography
Eosinophilia pathology
Granulomatosis with Polyangiitis drug therapy
Granulomatosis with Polyangiitis immunology
Humans
Hypertrophy, Left Ventricular diagnostic imaging
Hypertrophy, Left Ventricular etiology
Hypertrophy, Left Ventricular physiopathology
Immunologic Factors therapeutic use
Immunosuppressive Agents therapeutic use
Magnetic Resonance Imaging
Middle Aged
Stroke Volume
Churg-Strauss Syndrome complications
Eosinophilia complications
Granulomatosis with Polyangiitis complications
Heart Diseases complications
Hypertrophy, Left Ventricular drug therapy
Ventricular Function, Left drug effects
Subjects
Details
- Language :
- German
- ISSN :
- 1438-8790
- Volume :
- 75
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Pneumologie (Stuttgart, Germany)
- Publication Type :
- Academic Journal
- Accession number :
- 32707588
- Full Text :
- https://doi.org/10.1055/a-1209-4292