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1.
M. Harada R. Kuwahara H. Yoshida O. Hashimoto M. Sakamoto Y. Koga T. Kano M. Sata 《Modern rheumatology / the Japan Rheumatism Association》2001,11(1):80-82
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 相似文献
2.
We describe a case of Churg-Strauss syndrome (CSS) presenting with a massive pericardial effusion without overt myocardial
dysfunction. A 60-year-old man was referred to our hospital because of exertional dyspnea and fever. Initial chest multidetector-row
computed tomography showed a massive pericardial effusion. The presence of eosinophilia, infiltrates of both lungs, pathological
evidence of necrotizing vasculitis associated with eosinophilic infiltration, and history of asthma fulfilled the criteria
of CSS. Massive pericardial effusion can be the first manifestation of cardiac involvement in CSS. 相似文献
3.
Kensuke Ochi Yukio Horiuchi Kenichi Tazaki Kazuo Nishi Hidekazu Kawashima Hiroki Yabe 《Modern rheumatology / the Japan Rheumatism Association》2010,20(5):514-517
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. 相似文献
4.
Churg–Strauss syndrome (CSS) in a patient receiving pranlukast 总被引:2,自引:0,他引:2
Kobayashi S Ishizuka S Tamura N Takaya M Kaneda K Hashimoto H 《Clinical rheumatology》2003,22(6):491-492
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 相似文献
5.
Ana Sofia Correia Alexandra Gonçalves Vítor Araújo João Almeida e Silva José Manuel Pereira Pedro Rodrigues Pereira Manuel Pizarro João Carlos Silva Maria Júlia Maciel 《Revista portuguesa de cardiologia》2013,32(9):707-711
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. 相似文献
6.
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). 相似文献
7.
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
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.
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
10.
Jose Maria Bastida Monica Del Rey Nuria Revilla Rocio Benito Martin Perez-Andrés Berta González 《Platelets》2017,28(4):417-420
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. 相似文献
11.
《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
12.
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
13.
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
14.
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 相似文献
15.
Ming-xian ChenBi-lian Yu MD Dao-quan PengSheng-hua Zhou MD 《Heart & lung : the journal of critical care》2014
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. 相似文献
16.
《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
17.
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
18.
Wojciech Szczeklik Barbara Sokołowska Lucyna Mastalerz Piotr Grzanka Jacek Górka Karolina Pacułt Tomasz Miszalski-Jamka Jerzy Soja Jacek Musiał 《Clinical rheumatology》2010,29(10):1127-1134
Churg–Strauss syndrome (CSS) is a rare, systemic, necrotizing, small- and middle-sized vessel vasculitis which is accompanied
by blood eosinophilia, eosinophil infiltration of various tissues, and bronchial asthma. The lungs are the organs most often
involved in CSS. The aim of this study was a retrospective evaluation of the pulmonary findings in chest X-rays and high resolution
computed tomography (HRCT) in CSS patients at the time of initial diagnosis and to determine their frequency, character, and
location. Seventeen CSS patients were studied (12 women; 5 men; aged 29–56 years). In all patients at the time of initial
diagnosis, chest X-rays were performed, and in 15 patients, HRCT was performed additionally. The radiological images were
evaluated independently by two radiologists who reached a decision by consensus. Out of 17 patients studied, chest X-rays
revealed parenchymal abnormalities in 11, pleural effusion in three, and bronchial wall thickening in one. In five patients,
no abnormalities in chest X-rays were found. In HRCT, abnormalities were found in all patients (15 patients, 100%). Predominant
HRCT findings consisted of: ground-glass opacities and consolidations found in 13 patients (86.7%). Additionally, in four
patients, pulmonary micronodules were described; in ten, interlobular septal thickening; in three, linear opacities; in ten,
bronchial wall thickening and/or bronchial dilatation; and in three, pleural effusions. Ground-glass opacities and consolidation
abnormalities distribution pattern were peripheral in seven and random in six patients. In patients with CSS, the most common
pulmonary radiological findings are parenchymal opacities, which may be peripheral or random in distribution. Pathologic changes
were found in 70.6% of patient in chest X-rays, and in 100%, when HRCT was performed. These changes are nonspecific; however,
they should be not overlooked, as they may help in establishing the diagnosis and suggest the prognosis. 相似文献
19.
Churg–Strauss syndrome (CSS) is a rare illness with clinical findings characterized by asthma, eosinophilia, and vasculitis
affecting medium and small-sized arteries and veins in a variety of organs. Involvement of the temporal arteries by non-giant
cell eosinophilic vasculitis in CSS is quite rare and has only been published as isolated case reports or small patient series.
Myocardial infarction due to coronary artery vasospasm is an unusual manifestation of CSS. We describe a case of a 39-year-old
woman who had two myocardial infarctions due to severe coronary artery vasospasm and was diagnosed with CSS based on a temporal
artery biopsy. During the course of her treatment, she also had another rare manifestation of CSS, monocular blindness reversible
with immunosuppressive therapy. 相似文献
20.
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