Churg-Strauss Syndrome

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An Abdominal Presentation of Churg-Strauss Syndrome

An Abdominal Presentation of Churg-Strauss Syndrome

Churg-Strauss syndrome is a small and medium vessel vasculitis that is also known as allergic granulomatous angiitis. It most commonly presents with an asthma like symptoms. It was first described in Mount Siani Hospital, New York in 1951 by Jacob Churg and Lotte Stauss and was recognised after the study of a series of 13 patients who had asthma, eosinophilia, granulomatous inflammation necrotising systemic vasculitis and necrotising glomerulonephritis. We describe a case of Churg-Strauss syndrome presenting with abdominal pain and later during the hospital admission a mono-neuritis multiplex syndrome affecting the lower limbs. The patient presented in such an atypical fashion with abdominal signs and symptoms that they required laparotomy and the diagnosis was made after histological examination of tissue taken at the time of surgery. Treatment with immunosuppression and aggressive rehabilitation achieved a progressive recovery which continued on discharge from hospital.
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Churg-Strauss Syndrome in Children: A Clinical and Pathologic Review

Churg-Strauss Syndrome in Children: A Clinical and Pathologic Review

Churg-Strauss syndrome is a vasculitis accompanied by asthma and eosinophilia. It is generally considered a disease of adults; occurrence in children has been reported infrequently. Here we report 2 pediatric patients with Churg- Strauss syndrome manifesting with prominent pulmonary involvement. One, a 16-year-old with a previous history of asthma, presented with pleuritic chest pain and a peripheral pulmonary nodule complicated by an eosinophilic pleural effusion. The other patient presented at age 6 with cough, weight loss, and radiographic infiltrates. Lung biopsies revealed elements characteristic of Churg-Strauss syndrome, including eosinophilic microabscesses and vasculitis. Three- and 5-year follow-up showed continued symptoms in both patients despite medical therapy. Both patients illustrate many of the typical features of Churg-Strauss syndrome. We report these cases to expand the scant knowledge about Churg-Strauss syndrome in pediatric patients and to heighten awareness that this serious disease may affect the pediatric population. The relevant literature on Churg-Strauss syndrome, with specific reference to childhood cases, is reviewed.
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CHURG STRAUSS SYNDROME : A RARE CASE REPORT AND A BRIEF REVIEW OF LITERATURE

CHURG STRAUSS SYNDROME : A RARE CASE REPORT AND A BRIEF REVIEW OF LITERATURE

Churg-Strauss syndrome (CSS) is a rare primary, non-inheritable, non-transmissible systemic disease, which occurs in patient with history of bronchial asthma. CSS is an eosinophil-associated, small vessel granulomatous vasculitis, characterized by late onset asthma, upper airway disease, eosinophilia, and clinical manifestations of systemic vasculitis. We report a rare case of Churg-Strauss syndrome. A 48-year-old male diagnosed case of bronchial asthma presented with history of progressive dyspnoea, fever, productive cough. He responds rapidly to corticosteroids.
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Diffusion tensor imaging of peripheral nerves in Churg-Strauss syndrome

Diffusion tensor imaging of peripheral nerves in Churg-Strauss syndrome

Corresponding to this, on DTI tractography of the peripheral nerves, the common peroneal nerve was not visualized and the tibial nerve was poorly visualized at the right mid-thigh and popliteus area. Although we did not measure fraction anisotropy or diffusivity in our patient, these findings most probably reflect nerve injury 4,5 due to vasculitis. Recently, DTI has been applied to the study of peripheral nerves, e.g., median (carpal tunnel syndrome) 6, ulnar 6 , radial 7 , and tibial nerves 8 . These studies, including ours, demonstrate the feasibility of performing DTI of peripheral nerves to help evaluate peripheral nerve lesions. We here describe a case of a woman with asthma,
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Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome) presenting as diffuse myositis

Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome) presenting as diffuse myositis

Our case is unusual in that to our knowledge it is the first to report histologic features of primary inflamma- tory myopathy, such as phagocytosis of non-necrotic fi- bers and sarcolemmal overexpression of MHC-1, in a patient with EGPA presenting with myalgia and weak- ness. These findings raise the possibility that muscle involvement in our patient may not be merely the conse- quence of ischemia caused by middle-sized artery vascu- litis in muscle and nerve but may actually involve auto- immunity against muscle fibers. Co-occurrence of systemic vasculitis and true myositis is known to occur in overlap syndromes and mixed connective tissue diseases which generally involve a combination of lupus, scleroderma, Sjögren syndrome, rheumatoid arthritis and polymyositis, but these are not commonly associated with EGPA. In most multisystemic auto-immune diseases, specific organ involvement can vary significantly for reasons difficult to establish. Thus, there may not be a characterizable path- ophysiologic trigger for the occurrence of true myositis in EGPA.
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Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome)

Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome)

Abbreviations AAV: ANCA-associated vasculitides; ANCA: Antineutrophil cytoplasmic antibody; AZA: Azathioprine; BVAS: Birmingham vasculitis activity score; CNS: Central nervous system; CR[r]

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Identification of target antigens of anti endothelial cell and anti vascular smooth muscle cell antibodies in patients with giant cell arteritis: a proteomic approach

Identification of target antigens of anti endothelial cell and anti vascular smooth muscle cell antibodies in patients with giant cell arteritis: a proteomic approach

Serum samples were obtained from 15 patients who ful- filled the American College of Rheumatology (ACR) cri- teria for GCA [4] and 33 patients with anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis who fulfilled the ACR and the Chapel Hill criteria used as vasculitis controls, with the control group comprising 15 patients with Wegener’s granulomatosis (WG), 9 with Churg-Strauss syndrome (CSS) and 9 with microscopic polyangiitis (MPA) [18]. In each group of patients with ANCA-associated vasculitis, two-thirds of the patients had active disease as assessed by a Birmingham Vasculi- tis Activity Score (BVAS) >3 in the absence of treat- ment, and one-third of the patients had inactive disease as assessed by a BVAS <3. Some patients in both groups either received corticosteroids and/or immunsuppres- sants at the time of blood sampling. Sera from 12 healthy blood donors were used as healthy controls
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Sigmoid colon perforation in the patient with granulomatosis with polyangiitis

Sigmoid colon perforation in the patient with granulomatosis with polyangiitis

There is published literature on gastrointestinal in- volvement in systemic necrotizing vasculitis. Earlier re- ports showed that GPA patients presented with diarrhea, hematochezia, and gastrointestinal ulcers [5–7]. Pagnoux et al. reported the outcome of gastrointestinal involve- ment in 62 patients with systemic necrotizing vasculitis which include GPA, polyarteritis nodosa, Churg-Strauss syndrome, microscopic polyangiitis, and rheumatoid arthritis-associated vasculitis [5]. In their report, 9 of 62 patients (15%) had bowel perforation; however, there was no patient with GPA in 9 patients and the longest interval from diagnosis in their cohort was only 68 months. Furthermore, less than 20 cases of bowel per- foration associated with GPA were reported in published literature in English [8–21]. Table 1 shows the clinico- pathological features of the 15 reported cases [8–21] as well as those of the present case. The median patient age was 44 years (range, 19–69 years), and the male-to- female ratio was 11:4. The reported perforation sites were the jejunum in 2 cases, the ileum in 10 patients, and the colon in 4 patients. Histological analysis of the lesions revealed 9 and 4 instances of vasculitis and ulcer- ation, respectively. To the best of our knowledge, ours is the second case of isolated colon perforation. There were no reports about colon perforation with GPA pa- tients after rituximab administration without our patient.
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JOURNAL OF CLINICAL AND DIAGNOSTIC RESEARCH

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Indeed, severe GI involvement has been identified as an independent factor associated with poor outcome.[7] Considering the high risk of perforation of the GI tract, including the small intestine and the poor prognosis, any abdominal pain or discomfort must be carefully investigated. The Churg-Strauss syndrome (CSS), is a multisystem disorder characterized by allergic rhinitis, asthma, and prominent peripheral blood oeosinophilia[3]. Blood oeosinophilia (more than 10,000/mm3) is present in 95% of cases. Myeloperoxidase- specific antineutrophilic cytoplasmic antibodies (MPO-ANCA) are detected in 70% of patients with active Churg-Strauss syndrome, and may play an important role in the pathogenesis of the disease.[8]
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Kampo medicines improved blood test and QOL in two vasculitis cases of Churg&ndash;Strauss syndrome and Henoch&ndash;Sh&ouml;nlein purpura after inadequate treatment with conventional therapies

Kampo medicines improved blood test and QOL in two vasculitis cases of Churg&ndash;Strauss syndrome and Henoch&ndash;Sh&ouml;nlein purpura after inadequate treatment with conventional therapies

ChurgStrauss Syndrome (CSS) and Henoch–Shönlein purpura (HSP) belong to the vasculitis syndromes, in which inflammation and blood vessel damage are common. Since the blood vessels are normally responsible for sending adequate nourishment to the bodily tissues and the interior of these vessels is easily damaged, various patholo- gies result from such vessel damage. 1

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T helper cells as new players in ANCA associated vasculitides

T helper cells as new players in ANCA associated vasculitides

Anti-neutrophil cytoplasmic autoantibody (ANCA)- associated vasculitides (AAV) constitutes a group of disorders characterized by autoimmune necrotizing in- fl ammation of small blood vessels, which leads to systemic organ damage [1]. Th is group of systemic vasculitides includes Wegener’s granulomatosis (WG), microscopic polyangiitis (MPA), and ChurgStrauss syndrome (CSS). Th ese disorders are predominantly associated with the presence of circulating ANCAs that are directed against proteins in the cytoplasmic granules of neutrophils. ANCAs with specifi city for proteinase-3 (PR3-ANCA) are associated with WG to a high degree, whereas ANCAs with specifi city for myeloperoxidase (MPO-ANCA) are predominant in MPA and to a lesser degree in CSS [2]. Although it remains unknown how these conditions develop, it has been postulated that
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Familial Eosinophilic Granulomatosis with Polyangiitis

Familial Eosinophilic Granulomatosis with Polyangiitis

Eosinophilic Granulomatosis with Polyangiitis (EGPA) formerly known as Churg-Strauss syndrome (CSS) is a vasculitic syndrome characterized by sys- temic necrotizing vasculitis of small vessels with extravascular granulomas oc- curring among patients with asthma and tissue eosinophilia and is included in spectrum of ANCA associated vasculitis (AAV) disorders [1] [2] [3]. Peripheral neuropathy is the chief neurological complication affecting almost 70% of EGPA patients [4] [5]. It is a rare disease occurring with a prevalence of 10.7 to 14 per 1 million adults [6]. Familial EGPA cases are extremely rare and the only two ar- ticles found in literature comprised of a small number of cases [7] [8].
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Varied presentations of cutaneous vasculitis: a case series

Varied presentations of cutaneous vasculitis: a case series

Vasculitis involves a wide spectrum of clinicopathological process with reactive damage to the involved blood vessels. There is loss of vessel integrity instigating haemorrhage & luminal compromise leading to ischemia and necrosis of the tissue supplied by the involved vessels. It may affect varied size and type of blood vessels at different locations. It may be primary or secondary to systemic disease. It may involve a single organ like skin or may involve different organ systems at the same time. This case series include six cases of cutaneous vasculitis affecting different organs with varied presentations. Skin biopsies of six patients with unusual presentations were studied. Their complete history, physical examinations, laboratory investigations including serology were analysed and correlated with histopathological findings. The patients presented with different duration of symptoms varying from as short as 15 days to 1 year. Skin lesions were present in all cases while cardiac manifestation was seen in one. Serology and autoimmune disease markers were negative in all cases except one. However, histopathological features were in concordance with the clinical diagnosis of vasculitis. They were further classified as vasculitis secondary to Churg Strauss syndrome, venous stasis, Henoch Schonlein purpura or leucocytoclastic vasculitis.Vasculitis though a rare disease may manifest as an acute or chronic condition. It needs timely diagnosis by histopathological examination to aid in further management. It is important to assess the clinical severity in primary and secondary vasculitis, as it determines morbidity and mortality.
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Clinical review: Vasculitis on the intensive care unit – part 1: diagnosis

Clinical review: Vasculitis on the intensive care unit – part 1: diagnosis

The first part of this review addresses the diagnosis and differential diagnosis of the primary vasculitides Wegener’s granulomatosis, microscopic polyangiitis, ChurgStrauss syndrome and polyarteritis nodosa. Prompt diagnosis and treatment of these conditions ensures an optimal prognosis. The development of assays for antineutrophil cytoplasmic antibodies has aided the diagnosis of Wegener’s granulomatosis and microscopic polyangiitis. However, even in cases where there is high clinical likelihood that these conditions are present, up to 20% may be antibody negative, whereas alternative diagnoses may be antibody positive. The final diagnosis rests on a balance of clinical, laboratory, radiological and histological features. The exclusion of alternative diagnoses is important in assuring appropriate therapy. Particular attention is paid to the more fulminant presentations of these conditions and the role of the critical care physician in their diagnosis and management.
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Can Pentraxin-3 be a Candidate Marker in the Follow-Up of the Patients With Behçet’s Disease?

Can Pentraxin-3 be a Candidate Marker in the Follow-Up of the Patients With Behçet’s Disease?

in patients with Churg-Strauss syndrome, Wegener's granulomatosis, and microscopic polyangiitis, systemic lupus erythematosus, rheumatoid arthritis, and CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia), PTX-3 levels were higher in patients with untreated vasculitis and lower in patients who underwent immunosuppressive treatments (p<0.005). Different from our study, PTX-3 levels did not correlate with CRP levels in patients with vasculitis. 11

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Psychometric properties and validation of a four-item version of the Strauss–Carpenter scale in bipolar disorder

Psychometric properties and validation of a four-item version of the Strauss–Carpenter scale in bipolar disorder

Carpenter 1974) and found that it was effective in pre- dicting transition to a first psychotic episode in patients at high risk of psychosis. This scale has been used in many studies on first psychotic episodes that included patients diagnosed either with schizoaffective disorder or bipolar disorder (Melle et  al. 2000; Castro-Fornieles et al. 2011; Evensen et al. 2012; Barbeito et al. 2014; Jor- dan et al. 2014; Parellada et al. 2015). However, although the scale has been translated into Spanish for its use in schizophrenia (Ahuir et al. 2009), it has not been trans- lated yet for its use in bipolar disorder, despite that Span- ish is the second-most spoken language in the world. The objective of this study was to assess the reliability and validity of the four-item Strauss–Carpenter prognostic scale in measuring functioning in patients with bipolar disorder in the Spanish population.
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INVESTIGATING THE TEACHING METHODS BASED ON THE DEVELOPMENT OF FUTURE STUDIES IN THE HIGHER EDUCATION IN AFGHANISTAN

INVESTIGATING THE TEACHING METHODS BASED ON THE DEVELOPMENT OF FUTURE STUDIES IN THE HIGHER EDUCATION IN AFGHANISTAN

According to Strauss and Corbin (2011), strategies are behaviours, actions, interactions, actions that are used in the normal way and how people manage positions in dealing with issues and for solving them, and by the emergence of these phenomena are a special phenomenon. The three main strategies to implement teaching methods based on the development of future studies in higher education are based on interview data: One of the important strategies was to pay attention to the philosophical foundations of future studies such as "Attention to the relative affairs, the orientation of ontology, epistemology and the value of future studies". Another important strategy is the use of critical futures research methods such as text mining, text-mining, Delphi method, environmental scanning, brainstorming, normative
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Computing the Enclosures Eigenvalues Using the Quadratic Method

Computing the Enclosures Eigenvalues Using the Quadratic Method

In this article, We compute the enclosures eigenvalues (upper and lower bounds) using the quadratic method. The Schrodinger operator (A) (har- monic and anharmonic oscillator model) has used as an example. We study a new technique to get more accurate bounds. We compare our results with Boulton and Strauss method.

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“Resemblances to archaeological finds”: Guido Boggiani, Claude Lévi Strauss and Caduveo body painting

“Resemblances to archaeological finds”: Guido Boggiani, Claude Lévi Strauss and Caduveo body painting

The 1937 exhibition catalogue was short, containing relatively little about the Caduveo. However, in Tristes tropiques, published in 1955, Claude Lévi-Strauss dedicates a whole chapter to them. In his idiosyncratic combination of travel diary and memoir, Lévi-Strauss recalls how a girl was being prepared to celebrate her puberty: ‘Her shoulders, arms and face were painted with elaborate patterns, and all the available necklaces were hung round her neck’. He then interrupts this account with a casual confession: ‘This, incidentally, may have been prompted less by ancient custom than by a desire to impress us.’ 91 He then goes on to comment on the Caduveo women’s insistence that he photograph their decoration in return for payment – perhaps a legacy of Boggiani’s practice. Anxious ‘not to waste’ precious photographic film, Lévi-Strauss would sometimes pretend to be taking pictures, while handing over the money. 92 Yet he still needed to keep a record of their patterns. Lacking Boggiani’s artistic ability, Lévi- Strauss asked the women to draw the patterns on the paper themselves, observing how their art did not depend ‘on the natural contours of the human face’. 93
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Sodium uptake in different life stages of crustaceans: the water flea
Daphnia magna Strauss

Sodium uptake in different life stages of crustaceans: the water flea Daphnia magna Strauss

Daphnia magna Strauss is a small hyper-regulating freshwater cladoceran showing a vigorous, active NaCl uptake (Holm-Jensen, 1948; Stobbart et al., 1977; Potts and Fryer, 1979; Bianchini and Wood, 2002; Bianchini and Wood, 2003; Glover and Wood, 2005; Glover et al., 2005). This NaCl uptake is essential to counteract the continuous ion loss to the hypo-osmotic medium. This situation is aggravated in daphnids because they show a high surface to volume ratio. In fact, we have previously demonstrated that the sodium uptake rate is dependent on the body size and clearly determines the sensitivity of freshwater animals to ionoregulatory toxicants such as metals (Bianchini et al., 2002a; Grosell et al., 2002). Daphnids have been shown to be the most sensitive aquatic organisms to both waterborne copper and silver after either acute or chronic exposure (Ratte, 1999; Bianchini et al., 2002b; Grosell et al., 2002). Furthermore, the mechanisms of acute and chronic toxicity of these metals are associated with an alteration of the whole-body Na + concentration as a consequence of a metal-induced inhibition of the whole-body Na + ,K + -ATPase activity (Bianchini
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