Churg–Strauss syndrome presenting with eosinophilic myocarditis: A diagnostic challenge

Churg–Strauss syndrome (CSS) is an unusual disease that presents as systemic vasculitis and peripheral eosinophilia in patients with an atopic constitution. Cardiac involvement is unusual and often not prominent on initial presentation, but is an important cause of morbidity and mortality in patients with CSS. We report the case of a young woman with severe acute myocarditis. Coronary arteriography demonstrated extensive focal vasculopathy, consistent with coronary vasculitis, and myocardial biopsy showed eosinophilic myocarditis. This presentation led to an initial diagnosis of CSS in this patient and appropriate therapy resulted in a spectacular remission of disease activity.     

Dextromethorphan for neuropathic pain with Churg–Strauss syndrome

A 48-year old man who had been treated with prednisolone, aminophylline, and pranlukast, a leukotriene-receptor antagonist, was diagnosed with Churg–Strauss syndrome based on the findings of asthma, eosinophilia, mononeuropathy, and extravascular eosinophils. Intractable neuropathic pain of the legs was successfully controlled with an N-methyl-d-aspartate receptor antagonist, dextromethorphan. We suggest that dextromethorphan receptor antagonists may be a useful treatment for the pain of neuropathy caused by vasculitis syndrome. Received: June 26, 2000 / Accepted: October 21, 2000     

Spontaneous anterior interosseous nerve palsy with Churg–Strauss syndrome

A 74-year-old woman diagnosed with Churg–Strauss syndrome (CSS) complained of difficulty in flexing her left thumb and index finger. Electromyography demonstrated isolated anterior interosseous nerve (AIN) palsy, with no other peripheral neuropathy. We diagnosed this case as spontaneous AIN palsy complicated with CSS, the first case of this kind. Surgical treatment should be considered if no sign of recovery is seen within six months of onset.     

Churg–Strauss syndrome associated with leukotriene receptor antagonists (LTRA)

Churg–Strauss syndrome (CSS) is a rare vasculitic disorder that generally occurs in patients with bronchial asthma. CSS is being increasingly recognized in asthmatic patients treated with leukotriene receptor antagonists. However, the nature of this relationship remains to be elucidated. The present report describes three asthmatic patients who developed clinical manifestations highly suggestive of CSS, although one patient lacked the presence of eosinophilia. The patient, however, exhibited biopsy-proven cutaneous necrotizing vasculitis, which improved after withdrawal of montelukast. The second patient presented with systemic constitutional signs including fever, malaise, arthralgias, clinical jaundice, peripheral blood eosinophilia, and biopsy-proven eosinophilic hepatitis. The third patient also had circulating eosinophilia, scleritis, and arthritis. All patients improved after discontinuation of the leukotriene receptor antagonist (montelukast).     

Churg–Strauss syndrome: a rare presentation with otological and pericardial manifestations: case report and review of the literature

Churg–Strauss syndrome (CSS) is a rare multisystem autoimmune disease characterized by diffuse eosinophilic infiltration and necrotizing vasculitis. There are typical manifestations of longstanding rhinosinusitis and polyposis but otological manifestations are rare and characterized by dense aural discharge, granulomatous eosinophilic infiltrate in the mastoid and middle ear with conductive hearing loss, and progressive sensory neural hearing loss—all of which are not responsive to conventional treatment. We describe the case of a 59-year-old man with a rare presentation of CSS that included chronic bilateral otitis media with hearing loss and life-threatening pericardial tamponade. Treatment with pericardiocentesis, prednisone and cyclophosphamide was beneficial and resulted in an improvement of the pericardial and otological manifestations. Early recognition and treatment of otological involvement in CSS is extremely important because of the dramatic response to corticosteroids which may prevent progression of hearing loss.     

Nephropathy,polyneuropathy, and gastroenteritis in a child with Churg–Strauss syndrome

Churg–Strauss syndrome (CSS) is a serious but rare pauci-immune vasculitis of small- and medium-sized blood vessels. It is commonly seen in association with bronchial asthma and/or allergic disorders. The syndrome is characterized by the presence of asthma, hypereosinophilia, and vasculitis in any part of the body. Vasculitis is often associated with significant distortion of normal functions. A rather severe case of CSS in an 8-year-old Nigerian girl with asthma and allergic rhinoconjunctivitis is reported. She presented with multiple morbidities, namely, vasculitic polyneuropathy and also nephritic–nephrotic syndrome that eventuated in acute renal failure after an onset of vasculitic gastroenteritis. Routine screening of all asthmatic patients for CSS is advocated.     

Two subtypes of Churg–Strauss syndrome with neuropathy: the roles of eosinophils and ANCA

The aim of this study was to clarify the differences in the pathogenesis of neuropathy between myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-positive and -negative patients with Churg–Strauss syndrome (CSS). Eight MPO-ANCA-positive and 14 MPO-ANCA-negative patients were included. In addition to the standard histology, nerve biopsies were examined, employing immunohistochemistry for eosinophil major basic protein and electron microscopy. The groups did not differ significantly in clinical profiles, including the peak disability score and number of blood eosinophils. In nerve biopsies, necrotizing vasculitis was found in 63% (5/8) of the ANCA-positive and 21% (3/14) of the ANCA-negative patients. Fibrinoid necrosis of vessel walls was noted in 4 ANCA-positive patients (50%), and in one ANCA-negative patient (p = 0039). In contrast, a large number of eosinophilic infiltrations in the epineurium was shown in 36% (5/14) of the ANCA-negative patients, with no eosinophilic infiltrations shown in ANCA-positive patients. In 3 ANCA-negative patients, endoneurial eosinophils were seen where focal axonal loss and capillary dilatation were occasionally noted. There may be 2 pathogenetic mechanisms of neuropathy with CSS: ANCA-related vascular fibrinoid necrosis, and a toxic eosinophilic effect on nerve fibers which is independent of ANCA. Therapy targeting activated eosinophils may be a possible treatment for intractable neuropathy of CSS.     

Churg–Strauss syndrome (CSS) in a patient receiving pranlukast

Pranlukast is a cysteinyl leukotriene receptor I antagonist (LTRAs) approved for treatment of asthma in Japan since 1995. Compared to other LTRAs, such as zafilukast and montelukast, only few cases with Churg-Strauss syndrome (CSS) have been reported in association with treatment with pranlukast. We describe a 17-year-old Japanese male patient who developed CSS with a 13 month history of mild asthma receiving pranlukast for 11 months without systemic and/or inhaled corticosteroid administration prior to development of CSS. From the aspect of temporal relationship between treatment with pranlukast and development of CSS, a direct induction of CSS by pranlukast is suggested in our case.Abbreviations ANCA Antineutrophil cytoplasmic antibodies - CSS Churg–Strauss syndrome - LTRA Leukotriene receptor I antagonist     

Eosinophilic myocarditis due to Churg–Strauss syndrome mimicking reversible dilated cardiomyopathy

A 41-year-old woman with a history of asthma arrived at the emergency room of our hospital with dyspnea. The electrocardiogram showed no specific results. Echocardiography defects revealed an obvious decrease in the left ventricular systolic function and enlargement of the left chamber. We initially considered her condition to be dilated cardiomyopathy. However, she had eosinophilia in the peripheral blood and elevated cardiac enzymes. The coronary angiography showed normal coronary arteries. Single photon emission computed tomography (SPECT) showed infiltrative myocardial disease. She was then diagnosed with eosinophil infiltrations. Combined with peripheral nerve injury and lung involvement, she was diagnosed as having Churg–Strauss syndrome. After initiating prednisone treatment, her eosinophilia and rising cardiac enzymes recovered to normal, and both her echocardiographic abnormalities and symptoms noticeably improved.     

A Case of Churg–Strauss Syndrome Presenting with Cortical Blindness

A 46-year-old woman with a sudden sight loss due to infarction of the occipital lobes is reported. The association of pulmonary disease, digital ischaemia, polyneuropathy and peripheral eosinophilia led to a diagnosis of Churg–Strauss syndrome. Her vision partially improved by a treatment with steroids and monthly i.v. cyclophosphamide. To our knowledge, this is the first case of CSS with a sudden loss of vision due to bilateral occipital infarction. Received: 3 November 1999 / Accepted: 10 February 2000     

Marked improvement of Churg–Strauss vasculitis with intravenous gamma globulins during pregnancy

Churg–Strauss syndrome (CSS) is an extremely rare disease, and even less common in women of childbearing age. Patients with severe disease or those who are un-responsive to corticosteroids are usually treated with cytotoxic drugs, especially cyclophosphmide. Intravenous immunoglobulin (IVIg) has became a promising, but not completely accepted, form of treatment for systemic vasculitis that is un-responsive to standard therapy. We report a case of a woman who presented with a CSS flare during pregnancy. Because of mono-neuritis, treatment with IVIg was instituted with successful results. Our case not only supports the beneficial effect of IVIg in CSS, but it also illustrates its successful and safe use in a patient who was pregnant. We discuss the indication of IVIg during the course of anti-neutrophil cytoplasm antigen (ANCA) vasculitis during the pregnancy.     

Postpartum Churg–Strauss syndrome with severe cardiac involvement: Description of a case and review of the literature

Churg–Strauss syndrome (CSS) is a rare small- or intermediate-vessel necrotizing vasculitis typically characterized by asthma, lung infiltrates, necrotizing granulomas, and hypereosinophilia. In this report, we describe the case of a 35-year-old woman who, during her third trimester of pregnancy, developed dyspnea and, after delivery, severe cardiac failure which required heart transplantation. Diagnosis of CSS was made after performing a myocardial biopsy. We have also undertaken a review of the English-language literature regarding previously reported cases of pregnancies in women suffering from Churg–Strauss syndrome with particular attention to those patients with cardiovascular involvement.     

Churg–Strauss syndrome and congenital factor Leiden thrombophilia as nontypical causes of intracardiac thrombosis

We present and discuss the case of a 26-year-old man suffering from Churg–Strauss syndrome (CSS). In our patient the disease severely affected both the heart and the lungs. Left ventricular dysfunction and intracardiac thrombosis, probably secondary to the cardiotoxic effect of eosinophils, were revealed by echocardiography. The risk of thrombosis was additionally increased by the coexistence of the factor V Leiden gene mutation. An opportunely established diagnosis allowed institution of an appropriate pharmacotherapy and subsequently to obtain a significant abatement of the clinical symptoms as well as the laboratory manifestations of the disease.     

Adult onset Still’s disease flared with pericardial effusion

Adult onset Still’s disease (AOSD) is characterized by spiking fevers, arthritis, rash, and involvement of multiple organs, and can be classified as self-limited, intermittent, and chronic disease groups. Cardiac manifestations include pericarditis and myocarditis. The case of this disease flared only with pericardial effusion is not reported. We describe a patient with adult onset Still’s disease who was flared with pericardial effusion without other AOSD-associated symptoms, and propose that pericardial effusion should be included as a feature of flare in the intermittent disease group of adult onset Still’s disease.     

Wiskott–Aldrich syndrome in a child presenting with macrothrombocytopenia

Wiskott–Aldrich syndrome (WAS) is a rare X-linked recessive disease resulting from variants in the WAS gene, characterized by a triad of immunodeficiency, eczema, and thrombocytopenia. Despite the fact that WAS is traditionally differentiated from immune thrombocytopenia (ITP) by small size of WAS platelets, in practice, microthrombocytopenia may occasionally not be present, and in certain cases, WAS patients exhibit some parallelism to ITP patients. We characterized one patient presenting with the classic form of the disease but increased mean platelet volume. Molecular studies revealed a novel hemizygous 1-bp deletion in WAS gene, c.802delC, leading to a frameshift and stop codon at amino acid 308 (p.Arg268Glyfs*40). Next-generation sequencing of a total of 70 additional genes known to harbor variants implicated in inherited platelet disorders did not identify additional defects. The pathogenesis of macrothrombocytopenia in this case is not known, but probably the coexistence of a still unidentified additional genetic variant might be involved.     

Cardiac rupture or pericardial effusion?

After cardiogenic shock, myocardial rupture is the leading cause of in-hospital death from acute myocardial infarction (AMI). When possible, rapid diagnosis must lead to an emergency surgical repair to prevent sudden death. However, in some cases, despite new imaging techniques, the diagnosis may be difficult to obtain and the decision whether or not to operate, difficult. In the present report we describe the challenging case of a patient presenting a sub-acute cardiac rupture three days after anterior AMI.     

Effect of delayed diagnosis on disease course and management of Churg–Strauss syndrome: a retrospective study

Delayed diagnosis in patients with Churg–Strauss syndrome (CSS) is largely attributed to the variable and nonspecific presentation of the disease’s initial symptoms. The aim of the study was to evaluate the effect of delayed diagnosis on the course of CSS. We conducted a retrospective study of 30 CSS patients followed up in our department. In each patient, we assessed the delay in CSS diagnosis (the time when patients already fulfilled four out of six of the American College of Rheumatology criteria and the diagnosis was not yet established), the disease activity at the time of diagnosis, and organ involvement during CSS course. A median value of 2 weeks was chosen as the cutoff point after which the diagnosis was considered as delayed. Sixteen patients were diagnosed before (group 1) and 14 patients after this cutoff point (group 2). In group 2, we found a higher Birmingham Vasculitis Activity Score at the moment of diagnosis (20.4 vs 25.1, p?<?0.05) and a more severe disease course, resulting in more frequent hospitalization rates (0.64 vs 2.26/year, p?<?0.00001), higher corticosteroids dose requirements (5.87 vs 11.57 mg/day converted to methylprednisolone, p?<?0.0001), and additional immunosuppressive therapy administration (56.2 vs 92.8 %, p?<?0.05) to maintain disease remission. All six perinuclear pattern of antineutrophil cytoplasmic antibobodies (pANCA)-positive patients (20 %) were found in group 1. Concluding, the delay in diagnosis of CSS of more than 2 weeks was found to be associated with a disease course that was more severe. The presence of the pANCA antibodies may occasionally facilitate establishment of the diagnosis.     

The impact of current health-related quality of life on future health outlook in patients with eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome)

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, autoimmune small and medium vessel vasculitis. EGPA is accompanied by asthma and involves mainly the blood vessels of the lungs, gastrointestinal system, and peripheral nerves; however, the skin, kidneys, and heart may be also affected. To investigate if patients with EGPA experience reduced health-related quality of life (HRQOL), and the effect of this parameter on their own perception of future health outlook. Twenty-six EGPA patients are in disease remission and completed a custom-designed questionnaire and the Medical Outcomes Study Short Form 36 (SF-36). Using the RAND method, eight HRQOL dimensions were calculated: general health, physical functioning, emotional role limitations, physical role limitations, social functioning, mental health, bodily pain, and vitality. Using norm-based scores, the HRQOL of patients was compared with that of the general population. EGPA patients had decreased HRQOL across all eight dimensions of the SF-36. Patients with higher mental component score felt more positive about their future health, while patients with low physical component score were likely not to feel negatively about their future health. Also, 36 % of older patients (>50 years) had a positive outlook compared to 47 % of younger patients (<50 years) and patients with a longer disease course were much less likely to have a positive outlook (30 % positive) than those with a shorter course (50 % positive). Although not statistically significant, these correlations warrant further investigation with a larger patient population. Despite being in disease remission, EGPA patients had decreased quality of life, which in turn influenced their perception of their future health outlook.