A Case: Churg-Strauss Syndrome With Cardiac Involvement

Nino Mikaberidze, MD; Michael D. Lockshin, MD

Disclosures

July 13, 2017

A 66-year-old woman with a past medical history of hypertension, hyperlipidemia, osteoarthritis, asthma, and recurrent sinusitis received frequent antibacterial treatment and was treated with an antifungal agent for possible aspergillus infection 2 months prior to presentation. She had experienced diarrhea, malaise, decreased oral intake, and lethargy for 1 day. Forty-five minutes prior to arrival in the emergency department, her husband found her cold and clammy, with a pulse but without respiration. He initiated CPR and emergency medical services continued it. By the time of arrival, the patient had received 45 minutes of CPR, six rounds of epinephrine, and defibrillation.

She was unresponsive. Laboratory studies showed leukocytosis with 29% eosinophils, and ECG showed ST segment elevation and depressions. A chest x-ray showed diffuse consolidation; transthoracic echocardiogram revealed a severe hypokinetic inferolateral wall and moderate mid-anterolateral and anterior wall hypokinesis. Chest CT demonstrated di use bilateral lung consolidation, and bronchoscopy showed diffuse airway erythema and pink, frothy secretions. Left heart catheterization revealed areas of aneurysmal dilation of coronary arteries, with a giant proximal left circumflex aneurysm measuring 1 cm, involving the first obtuse marginal origin with severe ectasia (Figure), and an aneurysmal segment in the proximal first diagonal branch. The right coronary artery showed moderate ectasia.

Figure. Coronary angiography showing a giant proximal left circumflex aneurysm measuring 1 cm, involving the first obtuse marginal origin.

Given the patient's history of recurrent sinusitis, asthma, eosinophilia, and coronary aneurysms, we diagnosed eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome, CSS) and cardiac involvement, and recommended high-dose corticosteroid treatment. The patient remained critically ill on life support, and then suffered uncal and transtentorial herniation, at which time her family requested withdrawal of life support. Autopsy revealed necrotizing vasculitis involving pericardial and intramyocardial vessels, as well as eosinophilic and giant cell inflammation of the heart and lungs, supporting the diagnosis of CSS. The left circumflex coronary artery aneurysm was attributed to this diagnosis.

Case Discussion

CSS is a rare form of systemic vasculitis characterized clinically by asthma, hypereosinophilia, and vasculitis, and pathologically by extravascular granulomas and necrotizing vasculitis of small vessels. CSS typically consists of three distinct phases. The initial phase starts with asthma, allergic rhinitis, and nasal polyposis; the second is a period of peripheral and tissue eosinophilia associated with pulmonary infiltrates; and the third is characterized by systemic vasculitis.

Involvement of the heart usually occurs in the third phase as vasculitic lesions in myocardium and the coronary vessels, causing (peri)myocarditis, heart failure, cardiac tamponade, myocardial infarction, or pericardial effusion, as in our patient. Myocardial damage is caused by vasculitis, leading to coronary occlusion due to the release of toxic mediators by activated eosinophils causing direct myocardial damage, or by replacement of the myocardium with granulomas and scar tissue.[1]

Eosinophil activation in CSS requires specific cytokine stimulation. This role is partly mediated by CSS patients' T cells, which predominantly exhibit an activated Th2 phenotype, resulting in the secretion of high levels of interleukin (IL)-4, IL-13, and IL-5. IL-5 is particularly essential for eosinophil activation, maturation, and survival. Possible cross-talk occurs between eosinophils and Th2-type lymphocytes in CSS via the secretion of IL-25, a potent Th2-response enhancer, by the eosinophils themselves.

This and other factors—for example, eosinophil-apoptosis impairment through high levels of soluble CD95—might account for the persistent eosinophilia in active CSS. Eotaxin-3, a chemokine highly secreted by endothelial and inflammatory cells in CSS patients' damaged tissues, seems to directly target eosinophils toward affected tissues. Th1-type lymphocytes that secrete cytokines, such as interferon-gamma and soluble IL-2 receptor, are possible inducers of granuloma formation.[2]

Cardiac involvement in CSS is not rare. Depending on the report, its frequency varies between 16% and 92%. Heart involvement in CSS patients who are in clinical remission is very common and is characterized by fibrosis and an active inflammatory process.[3] Endomyocardial disease (Loeffler endocarditis) may represent the most severe form of cardiac involvement, similar to other hypereosinophilic disorders. High eosinophil counts and a negative ANCA test are associated with cardiac disease.[4] Cardiac involvement is also responsible for a dismal prognosis; it is the major cause of morbidity in CSS patients and the primary cause of their mortality (48% of deaths).[1]

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