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1.
S. Ovadia I. Dror T. Zubkov A. Tanay D. Levy G. Zandman-Goddard 《Clinical rheumatology》2009,28(Z1):35-38
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. 相似文献
2.
《Modern rheumatology / the Japan Rheumatism Association》2013,23(5):519-523
AbstractA 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. 相似文献
3.
Asako K Takayama M Kono H Kikuchi H 《Modern rheumatology / the Japan Rheumatism Association》2011,21(5):519-523
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. 相似文献
4.
5.
Niels P. Riksen Helmut Gehlmann Annemarie E. Brouwer Marcel van Deuren 《Clinical rheumatology》2013,32(1):41-42
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. 相似文献
6.
Skrapari I Kagkelari E Charitatos E Pantelidaki C Gounaris T Sioula E 《Clinical rheumatology》2008,27(1):125-127
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. 相似文献
7.
Bhagirath KM Paulson K Ahmadie R Bhalla RS Robinson D Jassal DS 《Rheumatology international》2009,29(4):445-449
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. 相似文献
8.
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. 相似文献
9.
Olowu WA 《Clinical rheumatology》2007,26(5):831-835
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. 相似文献
10.
Barbara Sokołowska Wojciech Szczeklik Lucyna Mastalerz Paweł Kuczia Michał Wodkowski Edyta Stodółkiewicz Karolina Macioł Jacek Musiał 《Clinical rheumatology》2013,32(3):349-354
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. 相似文献
11.
Oka N Kawasaki T Matsui M Shigematsu K Unuma T Sugiyama H 《Modern rheumatology / the Japan Rheumatism Association》2011,21(3):290-295
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. 相似文献
12.
Filiz Özerkan M.D. Mustafa Akin M.D. Azem Akilli M.D. Hakan Kültürsay M.D. Serdar Payzin M.D. Cüneyt Türkoğlu M.D. 《The International journal of angiology》2000,9(3):176-177
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. 相似文献
13.
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. 相似文献
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. 相似文献
14.
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. 相似文献
15.
Veres K Papp K Lakos G Szomják E Szekanecz Z Szegedi G Soltész P 《Clinical rheumatology》2008,27(1):111-113
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. 相似文献
16.
17.
Cozzi Alessandra Papagrigoraki Anastasia Biasi Domenico Colato Chiara Girolomoni Giampiero 《Clinical rheumatology》2016,35(5):1377-1382
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... 相似文献
18.
Basaran M Sever K Kafali E Ugurlucan M Alpagut U Dayioglu E 《Annales de cardiologie et d'angeiologie》2005,54(2):86-88
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. 相似文献
19.
《Modern rheumatology / the Japan Rheumatism Association》2013,23(4):685-687
AbstractA 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. 相似文献
20.
Kampitak T 《Clinical rheumatology》2008,27(8):1073-1075
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. 相似文献