Eosinophilic granulomatosis with polyangiitis; a distinctive presentation with myocarditis and autoimmune haemolytic anaemia: case report
BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare type of vasculitis characterized by inflammation within small blood vessels or tissues that may cause damage to the lungs, heart, kidneys, and other organs. Here, we present a rare case of EGPA with cardiac involveme...
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Frontiers Media S.A.
2025-01-01
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| Series: | Frontiers in Cardiovascular Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fcvm.2024.1490735/full |
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| author | Doaa Abdulwahab Mohammed Ayish Fatma Ayish Asayel Khamjan Amal. H. Mohamed Nagla Abdalghani Osama Albasheer |
| author_facet | Doaa Abdulwahab Mohammed Ayish Fatma Ayish Asayel Khamjan Amal. H. Mohamed Nagla Abdalghani Osama Albasheer |
| author_sort | Doaa Abdulwahab Mohammed Ayish |
| collection | DOAJ |
| description | BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare type of vasculitis characterized by inflammation within small blood vessels or tissues that may cause damage to the lungs, heart, kidneys, and other organs. Here, we present a rare case of EGPA with cardiac involvement that presented with acute heart failure.Clinical findingsA 44-year-old woman with a history of bronchial asthma and sinusitis presented with fever, shortness of breath, fatigue, unintentional weight loss, and polyarthritis. Physical examination revealed bilateral basal crepitation and mononeuritis multiplex.DiagnosisThe peripheral blood smear revealed red blood cells of different sizes and shapes (dimorphic features), more eosinophils, low hemoglobin, and higher lactate dehydrogenase (LDH) levels. Cardiac magnetic resonance imaging (CMR) revealed global hypokinesia and features suggestive of myocarditis. Echocardiography showed a low ejection fraction of 25%. Thus, the patient diagnosed with EPGA and myocarditis presented with acute heart failure.InterventionsThe patient was administered high-dose corticosteroids (intravenous bolus methylprednisolone 500 mg for three days, followed by 1 mg/kg of prednisolone) and cyclophosphamide 750 mg intravenously.OutcomeAfter one months, the patient showed a marked improvement in clinical and laboratory parameters. The ejection fraction improved to 30%–40%, the eosinophil count returned to normal, and the haemolytic anaemia resolved. The patient was sent home and shifted to mycophenolate mofetil 1 g twice a day as maintenance therapy.ConclusionPatients with EGPA have a higher morbidity and mortality rate when they have cardiac involvement. The pathophysiological mechanism of cardiac involvement in EGPA warrants consideration of immunosuppressive therapy in addition to standard heart failure treatment. |
| format | Article |
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| institution | Kabale University |
| issn | 2297-055X |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Cardiovascular Medicine |
| spelling | doaj-art-ea142ece0ed34e4babf44c574d47105b2025-01-14T06:10:36ZengFrontiers Media S.A.Frontiers in Cardiovascular Medicine2297-055X2025-01-011110.3389/fcvm.2024.14907351490735Eosinophilic granulomatosis with polyangiitis; a distinctive presentation with myocarditis and autoimmune haemolytic anaemia: case reportDoaa Abdulwahab Mohammed Ayish0Fatma Ayish1Asayel Khamjan2Amal. H. Mohamed3Nagla Abdalghani4Osama Albasheer5Medical and Rheumatology Consultant, Head of Medical Department in Jazan General Hospital, Jazan, Saudi ArabiaInternal Medicine Department, Faculty of Medicine, Jazan University, Jazan, Saudi ArabiaPGY4 Internal Medicine Residency Program, King Fahad Central Hospital-Jazan, Jazan, Saudi ArabiaInternal Medicine Department, Faculty of Medicine, Jazan University, Jazan, Saudi ArabiaRespiratory Therapy Department, Faculty of Applied Medical Sciences, Jazan University, Jazan, Saudi ArabiaFamily and Community Medicine Department, Faculty of Medicine, Jazan University, Jazan, Saudi ArabiaBackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare type of vasculitis characterized by inflammation within small blood vessels or tissues that may cause damage to the lungs, heart, kidneys, and other organs. Here, we present a rare case of EGPA with cardiac involvement that presented with acute heart failure.Clinical findingsA 44-year-old woman with a history of bronchial asthma and sinusitis presented with fever, shortness of breath, fatigue, unintentional weight loss, and polyarthritis. Physical examination revealed bilateral basal crepitation and mononeuritis multiplex.DiagnosisThe peripheral blood smear revealed red blood cells of different sizes and shapes (dimorphic features), more eosinophils, low hemoglobin, and higher lactate dehydrogenase (LDH) levels. Cardiac magnetic resonance imaging (CMR) revealed global hypokinesia and features suggestive of myocarditis. Echocardiography showed a low ejection fraction of 25%. Thus, the patient diagnosed with EPGA and myocarditis presented with acute heart failure.InterventionsThe patient was administered high-dose corticosteroids (intravenous bolus methylprednisolone 500 mg for three days, followed by 1 mg/kg of prednisolone) and cyclophosphamide 750 mg intravenously.OutcomeAfter one months, the patient showed a marked improvement in clinical and laboratory parameters. The ejection fraction improved to 30%–40%, the eosinophil count returned to normal, and the haemolytic anaemia resolved. The patient was sent home and shifted to mycophenolate mofetil 1 g twice a day as maintenance therapy.ConclusionPatients with EGPA have a higher morbidity and mortality rate when they have cardiac involvement. The pathophysiological mechanism of cardiac involvement in EGPA warrants consideration of immunosuppressive therapy in addition to standard heart failure treatment.https://www.frontiersin.org/articles/10.3389/fcvm.2024.1490735/fulleosinophiliamyocarditisvasculitisautoimmune haemolytic anaemiaheart failure |
| spellingShingle | Doaa Abdulwahab Mohammed Ayish Fatma Ayish Asayel Khamjan Amal. H. Mohamed Nagla Abdalghani Osama Albasheer Eosinophilic granulomatosis with polyangiitis; a distinctive presentation with myocarditis and autoimmune haemolytic anaemia: case report Frontiers in Cardiovascular Medicine eosinophilia myocarditis vasculitis autoimmune haemolytic anaemia heart failure |
| title | Eosinophilic granulomatosis with polyangiitis; a distinctive presentation with myocarditis and autoimmune haemolytic anaemia: case report |
| title_full | Eosinophilic granulomatosis with polyangiitis; a distinctive presentation with myocarditis and autoimmune haemolytic anaemia: case report |
| title_fullStr | Eosinophilic granulomatosis with polyangiitis; a distinctive presentation with myocarditis and autoimmune haemolytic anaemia: case report |
| title_full_unstemmed | Eosinophilic granulomatosis with polyangiitis; a distinctive presentation with myocarditis and autoimmune haemolytic anaemia: case report |
| title_short | Eosinophilic granulomatosis with polyangiitis; a distinctive presentation with myocarditis and autoimmune haemolytic anaemia: case report |
| title_sort | eosinophilic granulomatosis with polyangiitis a distinctive presentation with myocarditis and autoimmune haemolytic anaemia case report |
| topic | eosinophilia myocarditis vasculitis autoimmune haemolytic anaemia heart failure |
| url | https://www.frontiersin.org/articles/10.3389/fcvm.2024.1490735/full |
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