scholarly journals Cardiac Involvement in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Disease)

2021 ◽  
Author(s):  
Rashid Saif Al Umairi ◽  
Khalid Al Manei ◽  
Fatma Al Lawati ◽  
Yaqoob Al Mahrouqi ◽  
Farida Al Balushi
Keyword(s):  
Cardiac Involvement ◽  
Granulomatosis With Polyangiitis ◽  
Immunosuppressive Treatment ◽  
Vascular Diseases ◽  
Management Plan ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Heart Patients ◽  
Chest Clinic ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss disease, is a rare vasculitis that affects small- to medium-sized vessels and has a propensity to involve the heart. Patients with cardiac involvement have a poor prognosis and usually require immunosuppressive treatment along with corticosteroids. Cardiovascular magnetic resonance (CMR) is a noninvasive diagnostic tool that can detect cardiac involvement and guide the management plan. Herein, we present the case of a 39-year-old man with a known history of bronchial asthma who was referred to the chest clinic at the Royal Hospital for further assessment of persistent lung parenchymal changes on chest computed tomography. Given the clinical context of the patient and the radiological findings, EGPA was suspected. Lung biopsy confirmed the diagnosis of EGPA. CMR was performed for further assessment, which confirmed cardiac involvement. The patient was started on prednisolone and azathioprine and showed significant radiological and clinical improvement. Keywords: Eosinophilic Granulomatosis with Polyangiitis, Vasculitis, Eosinophils, Vascular Diseases, ANCA-associated Vasculiti

La granulomatosi eosinofilica con poliangioite: dalla pelle al cuore

2020 ◽  
Vol 39 (9) ◽  
pp. 569-574
Author(s):  
Matteo Pavan ◽  
Anna Agrusti ◽  
Andrea Trombetta ◽  
Serena Pastore ◽  
Alberto Tommasini ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Immunosuppressive Treatment ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Eosinophilic Inflammation ◽  
Peripheral Eosinophilia ◽  
Churg Strauss Syndrome ◽  
History Of ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Key words: Churg-strauss syndrome, Eosinophilic granulomatosis with polyangiitis in childhood, Vasculitis, Asthma, Hypereosinophilia Background - Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, is an extremely rare systemic vasculitis in the paediatric population. The hallmarks of eosinophilic granulomatosis with polyangiitis are a long history of asthma and peripheral eosinophilia with eosinophilic inflammation that may involve several organs. Findings - The paper reports the clinical characteristics, courses, and outcomes of the four patients diagnosed with eosinophilic granulomatosis with polyangiitis at IRCCS Burlo Garofolo (Trieste, Italy) from 1996 to 2015. The mean age at diagnosis was 11.5 years. All the patients presented a history of asthma and peripheral eosinophilia at diagnosis. 3/4 of the children presented upper airway and pulmonary disease. Skin and heart involvement was present in half of the patients, whereas gastrointestinal and neurological symptoms were reported in 25% of the cases. When performed, tissue biopsy revealed eosinophilic inflammation in all the cases. Anti-neutrophil cytoplasmic antibodies were negative in 66% patients. One young child died shortly after presentation, one remitted after immunosuppressive treatment and two patients needed low-dose corticosteroid therapy to maintain the remission. Conclusion - Comparison with an updated review of the series and cases of childhood-onset eosinophilic granulomatosis with polyangiitis reported in the literature showed similar demographic characteristics, clinical features and outcomes. Cardiac disease represents the poorer prognostic factor, leading to the 60% of the deaths reported.

scholarly journals Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

2020 ◽  
Vol 66 (7) ◽  
pp. 904-907
Author(s):  
Gabriela Venade ◽  
Cátia Figueiredo ◽  
Catarina Almeida ◽  
Nídia Oliveira ◽  
Luis Costa Matos
Keyword(s):  
Complex Disease ◽  
Granulomatosis With Polyangiitis ◽  
Previous History ◽  
Specific Treatment ◽  
Disease Diagnosis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Churg Strauss Syndrome ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

SUMMARY Churg–Strauss syndrome, Eosinophilic granulomatosis with polyangiitis (EGPA), is a systemic vasculitis that affects small– to medium-sized vessels. It is rare and part of the Anti-neutrophil cytoplasm antibody-associated vasculitis (ANCA) group. We present a 37-year-old man, with a previous history of asthma, that was sent to the ED due to 2 weeks of productive cough, occasional dyspnea on exertion, fever (one week), asthenia, and anorexia. Upon physical examination, he was subfebrile and tachycardic. He had leukocytosis (17.00 x10^9/L) and eosinophilia of 20.0 % (3.4 X10^9/L), creatinine level of 1.5 mg/dL, subtle elevation on liver function tests and CRP of 10.82mg/dL. On Chest X-Ray, there was infiltrate on the right pulmonary base. Due to a strong suspicion of EGPA, he was started on 80mg of prednisolone from admission. ANCA MPO was positive, with the remaining auto-immune study negative. He underwent Thorax CT (under corticotherapy) without relevant changes, as well as bronchoalveolar lavage, without macroscopic signs of alveolar hemorrhage. Because of active urinary sediment, nephrotic proteinuria (6.5g/24h), and acute renal failure he underwent a renal biopsy, which revealed pauci-immune crescentic glomerulonephritis, with predominantly acute findings (in the context of ANCA-MPO Vasculitis – EGPA). After the biopsy, he received three 1g methylprednisolone pulses and was started on Cyclophosphamide. He remained asymptomatic and renal function was restored. This case highlights the importance of integrating all findings in one clinical scenario to prevent a more complex disease diagnosis, with a specific treatment, from being missed.

scholarly journals CARDIAC INVOLVEMENT IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: A CHALLENGING CASE REPORT

2021 ◽  
Author(s):  
Larissa Maria Oliveira Gonzaga ◽  
Ana Luisa Bagno de Almeida ◽  
Aurivan Essado Dantas ◽  
Carolina Ruas Freire Santos ◽  
Anna Carolina Faria Moreira Gomes Tavares ◽  
...  
Keyword(s):  
Case Report ◽  
Cardiac Involvement ◽  
Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis
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