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.     

Churg–Strauss syndrome complicated by central retinal artery occlusion: case report and a review of the literature

Abstract

A 68-year-old man was admitted with rapid visual loss. Churg–Strauss syndrome (CSS) was diagnosed, based upon the symptoms of asthma, eosinophilia, interstitial pneumonitis, and positive myeloperoxidase-anti neutrophil cytoplasmic antibody (MPO-ANCA). Light reflexes were absent and vision was completely lost in both eyes. Bilateral central retinal artery occlusion (CRAO) was observed by fluorescence angiography. Steroid pulse along with an anticoagulant improved the visual acuity to light perception and hand motion. CSS-associated CRAO should be considered when acute visual loss occurs.     

Churg–Strauss syndrome complicated by central retinal artery occlusion: case report and a review of the literature

A 68-year-old man was admitted with rapid visual loss. Churg–Strauss syndrome (CSS) was diagnosed, based upon the symptoms of asthma, eosinophilia, interstitial pneumonitis, and positive myeloperoxidase-anti neutrophil cytoplasmic antibody (MPO-ANCA). Light reflexes were absent and vision was completely lost in both eyes. Bilateral central retinal artery occlusion (CRAO) was observed by fluorescence angiography. Steroid pulse along with an anticoagulant improved the visual acuity to light perception and hand motion. CSS-associated CRAO should be considered when acute visual loss occurs.     

Complete remission of coronary vasculitis in Churg–Strauss Syndrome by prednisone and cyclophosphamide

The heart is involved in up to 50% of all patients with Churg–Strauss syndrome, but vasculitis of the coronary arteries has only been rarely documented. We present a young patient with severe coronary aneurysms and stenotic lesions due to a Churg–Strauss vasculitis. Prompt therapy with prednisone and cyclophosphamide resulted in the complete resolution of all lesions.     

Acute painless monocular visual loss due to central retinal artery occlusion in a patient with Churg–Strauss vasculitis

Ocular involvement in Churg–Strauss syndrome (CSS) is infrequent. We describe a case of a 50-year-old woman, with blood eosinophilia, involvement of the respiratory tract, skin, and peripheral nervous system, fulfilling the American College of Rheumatology criteria for CSS, who presented with left foot drop followed by left acute painless visual loss. Central retinal artery occlusion was diagnosed by fundoscopic findings (retinal whitening with a cherry-red spot). CSS was confirmed by sural nerve biopsy. Despite treatment with high-dose corticosteroids, cyclophosphamide, and anticoagulant therapy, visual acuity was not substantially improved. Acute blindness in CSS has been rarely described. Even more rarely, central retinal artery occlusion has been found to be the underlying cause of this infrequent clinical manifestation in CSS.     

Clinical utility of cardiac magnetic resonance imaging in Churg–Strauss syndrome: case report and review of the literature

We describe a case of an individual with Churg–Strauss syndrome who presented with a cerebrovascular accident (CVA) secondary to left ventricular intracavitary thrombi. Noninvasive cardiovascular imaging using transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) was used to identify the cardioembolic source of CVA. The clinical utility of CMR in the management of patients with Churg–Strauss syndrome is reviewed.     

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.     

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.     

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.     

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.     

Transcatheter embolization of coronary to pulmonary artery fistula with coronary steal phenomenon—a case report

Percutaneous transcatheter closure technique of a coronary artery fistula with a coil was performed in a 32-year-old man who had stable angina pectoris and myocardial ischemia in thalium myocard scintigraphy. Complete closure of the fistula without any complications was confirmed by angiography after the procedure. Angina pectoris disappeared and thalium scintigraphy was normal after the transcatheter embolization of fistula.     

Internal mammary artery steal syndrome secondary to a para-IMA after coronary artery bypass graft： a case report

Introduction
The use of the intemal mammary artery （IMA） in coronary artery bypass graft（CABG） for myocardial revascularization is gaining popularity in routine practice, especially when the target vessel is the left anterior descending artery （LAD）. Occasionally, IMA hypoperfusion occurs when there is inadequate flow through the IMA graft to the LAD artery due to the exist of lateral branches.     

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.     

Association of HELLP syndrome with primary antiphospholipid syndrome—a case report

Authors present the first Hungarian case of a young pregnant woman with the association of antiphospholipid syndrome (APS) and hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. After the onset of severe preeclampsia, the pregnancy was terminated but the patient’s condition continued to worsen. New symptoms of APS, including deep vein thrombosis and ischemic nervus opticus lesion, developed in the patient followed by the onset of acute respiratory distress syndrome, which required respiratory therapy. Intensive treatment with plasmapheresis, high-dose intravenous immunoglobulin, high-dose corticosteroids, cyclophosphamide, and anticoagulants eventually led to full recovery. There have been only few scattered reports in the literature on the association of HELLP syndrome and APS, which was successfully managed with the combination of various immunosuppressive and immunomodulatory treatment modalities.     

Cutaneous manifestations of adult-onset Still’s disease: a case report and review of literature

Clinical Rheumatology - Adult onset Still’s disease (AOSD) is a systemic inflammatory disorder of unknown etiology and pathogenesis characterized by high spiking fever, arthralgia or...     

The vascular manifestations of Behçet's disease: a case report

Behçet’s disease is an inflammatory vasculitis which affects the arteries and veins. The vascular pathologies are the rare complications of this disease. We present here a patient with Behçet’s disease who has been hospitalized several times because of plurifocal vascular manifestations.     

Successful treatment of eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg–Strauss syndrome) with rituximab in a case refractory to glucocorticoids,cyclophosphamide, and IVIG

Abstract

A 44-year old woman with eosinophilic granulomatosis with polyangiitis (EGPA) developed sequential paralysis of different cranial nerves despite treatments including methylpredonisolone pulse therapy, intravenous immunoglobulins (IVIG), and cyclophosphamide. Infusions of rituximab ameliorated her neurological symptoms and serological inflammatory findings. Rituximab, a specific B cell-targeting therapy, might offer an alternative for refractory EGPA with possible advantages of cost and ease of use compared to IVIG, which also targets (at least in part) B lymphocytes and immunoglobulin production.     

Fatal Kikuchi–Fujimoto disease associated with SLE and hemophagocytic syndrome: a case report

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare, benign, autoimmune condition characterized by lymphadenopathy, fever, and neutropenia. KFD can frequently mimic various diseases including infection, malignancy, and autoimmune disease. KFD has also been infrequently reported in association with SLE, which can be diagnosed previously, simultaneously, or after the diagnosis of SLE. The author presents here the first case of fatal KFD simultaneously occurred with SLE complicated with hemophagocytic syndrome and severe infection. The recognition of this association is of significance for management of affected patients with early intensive immunosuppressive therapy for favorable outcome.