Unusual cardiac involvement in granulomatosis with polyangiitis manifesting as acute congestive heart failure

Kyaw, Htoo; Misra, Deepika; Mani, Malary; Park, Won J.; Shinnar, Meir

doi:10.14744/anatoljcardiol.2017.7732

Cited by 6 publications

(7 citation statements)

References 8 publications

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“…Common cardiac manifestations are pericarditis, myocarditis, and conduction abnormalities [ 5 , 9 ]. DCM associated with GPA is very rare and has been reported in a few case reports since GPA was described in 1936 by Wegner [ 4 , 7 , 8 , 10 ]. The exact mechanism of cardiac failure is unknown.…”

Section: Discussionmentioning

confidence: 99%

“…Pericarditis and coronary arteritis are the most common cardiac manifestations, but left ventricular global systolic dysfunction, conduction abnormalities, and pericardial effusion have also been described [4,5]. Only four case reports of dilated cardiomyopathy (DCM) associated with GPA have been described [6][7][8]. The patients described in these reports had left ventricular systolic dysfunction with reduced ejection fraction.…”

Section: Introductionmentioning

confidence: 99%

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An Unusual Case of Dilated Cardiomyopathy in Wegner’s Granulomatosis

Chhetri¹,

Gole²,

Mallari³

et al. 2022

Cureus

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A 33-year-old male presented to the emergency with cough, hemoptysis, and shortness of breath. He was on steroids for suspected Still’s disease due to arthralgias and fever prior to presentation to the emergency. He developed sudden hypoxic respiratory failure and required mechanical ventilation. The initial imaging studies of the chest including computed tomography (CT) of the chest showed marked diffuse central and basilar predominant opacities with associated smooth septal thickening. Furthermore, the patient’s creatinine, troponin, B-type natriuretic peptide (BNP), rheumatoid factor, and D-dimer were elevated. Vasculitis workup, bronchoscopy, and echocardiogram were performed. The echocardiogram revealed severely decreased left ventricular systolic function with an ejection fraction of 24% with dilated left ventricle. The electrocardiogram did not show any findings of acute ischemia. He was started on pulse dose steroid and dobutamine drip along with intermittent diuresis. The patient was successfully extubated after two days of mechanical ventilation. He was started on cyclophosphamide in the hospital. Dobutamine was discontinued. He was moved to the general medical floor as his oxygenation improved, but later at night, he developed respiratory failure and required a bumetanide drip. The cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA) (anti-PR-3 antibody) came back positive with titer >1:40, so Wegner’s granulomatosis was diagnosed. He received three sessions of plasmapheresis. The patient’s kidney function improved significantly, and the bumetanide drip was transitioned to intravenous pushes. His oxygenation improved significantly with saturations of 92% on room air. The patient was discharged on steroid, Bactrim, and systolic heart failure medications to follow up with rheumatology, nephrology, pulmonology, and cardiology in the office. Due to insurance issues, his outpatient care was delayed significantly. The patient followed up with rheumatology after two months and has been planned for rituximab induction and to continue steroid along with Bactrim. This case is worth reporting because it describes dilated cardiomyopathy (DCM) as a cardiac manifestation of Wegner’s granulomatosis. Early cardiac evaluation should be incorporated into the management of the patient suspected of Wegner’s granulomatosis.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

An Unusual Case of Dilated Cardiomyopathy in Wegner’s Granulomatosis

Chhetri¹,

Gole²,

Mallari³

et al. 2022

Cureus

View full text Add to dashboard Cite

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“…Patients with GPA have an increased risk of venous thromboembolism (VTE), which includes deep vein thrombosis (DVT) and pulmonary embolism (PE), especially during times of active GPA disease [ 3 ]. Cardiac involvement is a rare complication of GPA with an estimated incidence of 3.3% [ 4 ]. Respiratory involvement is common due to the necrotizing granulomatous inflammation that is produced in the upper and lower airways [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

Granulomatosis With Polyangiitis: Cardiac, Renal, and Respiratory Involvement

Khatiwala,

Patel,

Nachodsky

2024

Cureus

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Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is an antineutrophilic cytoplasmic autoantibody (ANCA)-associated small-vessel vasculitis. Typically, it causes upper and lower respiratory tract necrotizing granulomatous inflammation and necrotizing glomerulonephritis. The diagnosis is made through clinical symptoms, positive antibody testing, imaging, and kidney biopsy. We describe the case of a man in his 60s who presented with multiple complications of GPA including rapidly progressive renal failure requiring dialysis, diffuse alveolar hemorrhage, acute respiratory distress syndrome (ARDS), circulatory shock, submassive pulmonary embolism, and biventricular and dilated cardiomyopathy.

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“…Granulomatosis with polyangiitis: Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis is among the rarest but potentially life-threatening systemic necrotizing vasculitis predominantly affecting small and medium sized vessels [110,111]. The upper and lower respiratory system, systemic vasculitis, and necrotizing glomerulonephritis are the characteristic components of the disease triad [112].…”

Section: Introductionmentioning

confidence: 99%

“…GPA can manifest in multiple organ systems and presents with a variety of symptoms. Cardiac involvement is rarely documented antemortem with an estimated incidence of 3.3% to 13% [111,115,116]. Besides increasing incidence of GPA, the difference in the incidence may also be due to heterogeneity of cohorts, different definitions of cardiac manifestations and different diagnostic tools used [117].…”

Section: Introductionmentioning

confidence: 99%

autoimmune-cardiomyopathy-a-review-and-pooled-analysis-of-pathophysiology-diagnosis-and-clinical-management

Albakri¹

2019

Med Clin Arch

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Dilated cardiomyopathy (DCM), characterized by dilatation of cardiac chambers and systolic dysfunction, is one of the leading causes of severe heart failure (HF) and the most common indication for heart transplantation. Despite significant therapeutic improvements, its incidence and mortality is persistently high largely attributable to heterogeneous aetiology. Infectious diseases and genetic factors predominate research on the causes of DCM. However, evidence over the past two decades reveal distinct autoantibodies or other immune factors in heterogeneous subsets of DCM suggesting the involvement of autoimmune mechanisms in the pathogenesis of DCM. Hitherto, accumulated evidence demonstrates abnormalities in cell-mediated immunity such as altered lymphocyte function and relative proportion of lymphocytes subsets, activated cytokine system, inappropriate expression of histocompatibility complex on cardiac tissue and the expression of adhesion molecules on cardiomyocytes. This evidence suggest a subset of DCM patients is autoimmunity-mediated. This review aims to summarize the contemporary understanding of the characteristics of autoimmune cardiomyopathy in humans, proposed pathophysiology mechanisms as well as the ongoing clinical investigations that have focused on autoimmunity as a target for therapy.

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Unusual cardiac involvement in granulomatosis with polyangiitis manifesting as acute congestive heart failure

Cited by 6 publications

References 8 publications

An Unusual Case of Dilated Cardiomyopathy in Wegner’s Granulomatosis

An Unusual Case of Dilated Cardiomyopathy in Wegner’s Granulomatosis

Granulomatosis With Polyangiitis: Cardiac, Renal, and Respiratory Involvement

autoimmune-cardiomyopathy-a-review-and-pooled-analysis-of-pathophysiology-diagnosis-and-clinical-management

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