Churg-Strauss Syndrome Patients Research Articles

A good visual recovery in a patient with central retinal artery occlusion associated with Churg-Strauss syndrome: case report

Background: Churg-Strauss syndrome (CSS) is a rare systemic necrotizing vasculitis of unknown etiology that affects small-to-medium-sized vessels. A multisystem disease that mostly affects the lungs and the skin. In addition, it could affect the renal, cardiovascular, gastrointestinal, central, and peripheral nervous systems. Ocular involvement in the CSS is rare. The reported ocular manifestations include corneal ulcer, uveoscleritis, conjunctival granuloma, orbital inflammatory pseudotumor, amaurosis fugax, retinal artery occlusion, ischemic optic neuropathy, oculomotor nerve palsy, and trochlear nerve palsy. Case Presentation: A 53-year-old man was admitted with sudden visual loss. CSS was diagnosed earlier, based on asthma, eosinophilia, peripheral neuropathy symptoms, and pulmonary infiltrates. His vision was completely lost in the left eye. Unilateral central retinal artery occlusion (CRAO) was observed by fluorescence angiography. Results: The treatment modality used in our patient was steroid pulse therapy, and his vision improved 24 hours later. This outcome might emphasize the importance of steroid pulse therapy since it may be effective for CRAO in CSS patients. Conclusion: This case report described a CSS patient with associated CRAO, in whom steroid pulse therapy improved his visual acuity. Therefore, since intensive treatment should be administered as early as possible to improve the prognosis of vision, rheumatologists should be aware of the presence of CSS-associated CRAO, and they should refer those patients to ophthalmology as soon as possible.

Comprehensive myocardial tissue characterization with cardiac magnetic resonance in patients with Churg Srauss Syndrome

Background Churg-Strauss syndrome (CSS) is a rare systemic necrotizing vasculitis characterized by asthma, hypereosinophilia, cardiac failure, renal damage and peripheral neuropathy. CSS typically develops in three clinical phases, beginning with asthma, followed by tissue eosinophilia and finally in a systemic small-vessel vasculitis. During the late phase, coronary vessels and myocardium are interested leading to a variety of clinical manifestations such as myocarditis, heart failure, pericarditis and coronary vasculitis. Heart involvement is found in over 50% of autopsied CSS patients with evidence of an extremely high mortality in those with cardiac disease left untreated. Cardiac MR has emerged as a promising technique not only for the early diagnosis of cardiac involvement in the setting of this insidious and complex condition, but also for a better risk stratification and tailoring of immunosuppressive therapy. Methods

Churg-Strauss syndrome: a new endotype of severe asthma? Results of 14 Turkish patients.

Churg-Strauss syndrome (CSS) is a rare multisystem vasculitis. Considering the variation of autoimmune diseases in different races, it is of interest to determine whether any outstanding features exist for Turkish patients with CSS. The aim of this study was to evaluate the clinical and serological features of the disease, the treatment, and long-term follow-up details, and to investigate possible etiological factors of Turkish CSS patients. The study included 14 patients who were diagnosed with CSS, and followed by our department between 2004 and 2012. Possible etiological factors, initial symptoms, clinical presentations, treatment, as well as outcomes were documented. The study was approved by the local ethics. All patients fulfilled the American College of Rheumatology criteria. Initial symptoms were worsening asthma (n = 14; 100%) and skin lesions (n = 6; 43%). All patients had a diagnosis of asthma and nasal polyps, whereas 57.1% had aspirin hypersensitivity at the time of diagnosis. The lungs (100%) and skin (43%) were most commonly involved. Peripheral eosinophilia dominated on initial presentations of all patients. Initial treatments included oral methyl prednisolone in all cases, whereas cyclophosphamide and azathioprine were used in three cases. Relapses were detected in five cases. None of the cases were able to stop the oral corticosteroid treatment. No fatalities were observed. We herein describe a new severe asthma endotype in connection with CSS. We suggest that physicians who deal with uncontrolled severe asthma cases should consider CSS in the presence of nasal polyps, aspirin hypersensitivity, and especially peripheral blood eosinophilia over 10%.

Clinical features and prognostic factors of Churg-Strauss syndrome

Background/AimsChurg-Strauss syndrome (CSS) is a rare systemic necrotizing small-vessel vasculitis, with accompanying bronchial asthma, eosinophilia, and eosinophilic infiltration of various tissues. The purposes of our study were to characterize the clinical features of CSS and to identify factors associated with CSS prognosis in Koreans.MethodsMedical records were reviewed retrospectively for all physician-diagnosed CSS patients in the Seoul National University Hospital between January 1990 and March 2011.ResultsData from 52 CSS patients were analyzed. The respiratory tract was the most commonly involved organ (90.4%). Renal involvement was less frequent in antineutrophilic cytoplasmic antibody (ANCA)(-) patients than in ANCA(+) patients (p = 0.048). Clinical remission occurred in 95.3% of patients, but 16.3% of them relapsed. Patients who maintained remission for more than 6 months were relatively older (median, 51 years) at diagnosis (p = 0.004), had been diagnosed in earlier stages (p = 0.027), showed more frequent respiratory involvement (p = 0.024) and generalized symptoms (p = 0.039), and showed less frequent cutaneous involvement (p = 0.030) than those who did not achieve persistent (> 6 months) remission. Patients who achieved persistent remission also showed higher C-reactive protein (CRP) levels (p = 0.031) than those who did not.ConclusionsANCA(-) CSS patients showed less frequent renal involvement. Characteristics of good responders were older age, diagnosis at earlier stages, less cutaneous involvement, more respiratory involvement, high CRP values, and more generalized symptoms.

Churg-Strauss sindromas

Churg-Strauss syndrome (CSS) is a rare, systemic necrotising small and medium size vessel vasculitis of unknown origin. It was described initially by Churg and Strauss in 1951. It is now classified as an antineutrophil cytoplasmic antibody (ANCA) associated vasculitis. CSS specific signs, which differentiate this syndrome from other small vessel vasculites are late onset asthma, extravascular necrotising granulomas, blood and tissue eosinophilia. According to literature, skin changes are seen in more than half of CSS patients and in some cases are the first signs of the disease. Symptoms of skin or other organ damage and skin biopsy results should alert clinicians about CSS presence and may help to prescribe appropriate etiopathogenetic treatment. This article presents a case report of rare CSS and a literature review. Article in Lithuanian

THU0205 The temporal appearance of symptoms and signs in churg strauss syndrome: From the prodromal phase to damage

Objectivesto characterise the clinical presentation of Churg Strauss syndrome (CSS) in a monocentric cohort of patients and to assess the response to treatment and the long-term outcome of the disease.MethodsWe...

SAT0166 The Incidences of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in Northeastern Part of Turkey.

BackgroundThe epidemiological findings of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) differ geographically. Until now, there has been no study about the prevalence of AAV in Turkey.ObjectivesIn our study, we aimed to...

AB0049 Eotaxin is overexpressed in churg-strauss syndrome compared to allergic asthma

BackgroundHistorically, allergic asthma has been considered the background of the development of Churg-Strauss Syndrome (CSS)1,2. Recently, a retrospective study comparing CSS and allergic asthma proved that respiratory atopy or allergy,...

A case of Churg-Strauss syndrome and central retinal artery occlusion with good visual recovery

Here we report a case of Churg–Strauss syndrome (CSS) and central retinal artery occlusion (CRAO), with good visual recovery. A 58-year-old Japanese man with CSS experienced acute painless loss of vision in his right eye. CRAO was diagnosed by fundoscopic findings (retinal whitening with a cherry-red spot). Steroid pulse therapy (methylprednisolone at 1 g daily for 3 days) followed by combined treatment with prednisolone (30 mg/day) and cyclophosphamide (150 mg/day) was administered; his visual acuity recovered to 20/30 in 1 month, and no recurrence has occurred for 1 year. Steroid pulse therapy may be effective for CRAO in CSS patients.

A Case of Churg–Strauss Syndrome: Flow Cytometric Analysis of the Surface Activation Markers of Peripheral Eosinophils

© 2013 The Authors. doi: 10.2340/00015555-1384 Journal Compilation © 2013 Acta Dermato-Venereologica. ISSN 0001-5555 Churg–Strauss syndrome (CSS) is a complex multiple organ disease (1). The American College of Rheumatology has proposed 6 criteria for CSS classification, with the fulfilment of 4 criteria making a diagnosis of CSS (2). These criteria include asthma, eosinophilia, mononeuropathy or polyneuropathy, pulmonary infiltrates, paranasal sinus abnormality and extravascular eosinophils. Cutaneous manifestations are frequently observed in 40% of CSS patients. We report here a case of CSS presenting atypical cutaneous and subcutaneous manifestations of CSS with flow cytometric analysis of the peripheral eosinophils.

Standard and feature tracking magnetic resonance evidence of myocardial involvement in Churg–Strauss syndrome and granulomatosis with polyangiitis (Wegener’s) in patients with normal electrocardiograms and transthoracic echocardiography

The aim of the study was to evaluate the presence and spectrum of cardiac abnormalities identified by cardiac magnetic resonance (CMR) in subjects in clinical remission of Churg–Strauss syndrome (CSS) and granulomatosis with polyangiitis (Wegener’s) (WG) with normal ECG and transthoracic echocardiography (TTE). Eleven (7 females, 4 males, mean age 42.4 ± 9.6 years) CSS and 10 (4 females, 6 males, mean age 45.3 ± 10.9 years) WG patients in clinical remission with normal ECG and TTE underwent CMR. Segmental peak-systolic myocardial strain (εps) was measured using feature tracking cine-sequence based technique. Left ventricular (LV) ejection fraction, end-diastolic volume and myocardial mass indexes were 66.2 ± 5.8 %, 66.1 ± 6.6 ml/m2, and 61.0 ± 8.9 g/m2, respectively. No patient showed regional wall motion abnormalities and signs of myocarditis. Nine CSS and 8 WG patients demonstrated decreased segmental longitudinal, circumferential or radial εps and myocardial late gadolinium enhancement (LGE) (6 subendocardial, 10 midwall, 8 subepicardial) areas. In CSS and WG subjects with LVLGE lesions the mean LVLGE extent was 2.0 ± 1.6 % and 2.3 ± 1.5 % (p = 0.65), respectively. Segmental εps was decreased longitudinally (−11.8 ± 5.6 %) for subendocardial LGE, radially (13.7 ± 8.7 %) for subepicardial LGE, and circumferentially (−16.6 ± 4.2 %), longitudinally (−13.2 ± 5.5 %) and radially (18.8 ± 8.1 %) for midwall LGE, if compared to longitudinal (−22.7 ± 5.1 %), circumferential (−23.6 ± 5.6 %) and radial (34.2 ± 15.7 %) εps in controls (11 females, 10 males, mean age 43.9 ± 10.5 years) (all p < 0.01). Despite clinical remission, normal ECG and TTE, most CSS and WG patients demonstrate decreased segmental εps and non-ischemic LGE lesions without signs of myocarditis.

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.

Churg&amp;ndash;Strauss syndrome associated with antiphospholipid antibodies in a patient with recurrent myocardial and cerebral ischemia

We report on a case of Churg–Strauss syndrome (CSS) associated with the presence of antiphospholipid antibodies. The patient had a history of recurrent myocardial infarction and presented with acute ischemic cerebral disease. Eosinophilia with typical lung and skin lesions led us to diagnose the patient with CCS. We hypothesize that the presence of antiphospholipid antibodies significantly contributed to the ischemic events. We suggest that the search for antiphospholipid antibodies should be included in the laboratory work-up in CSS patients and patients affected by primary systemic vasculitides in general. Moreover, anticoagulant treatment appears to be warranted in all CSS patients and antiphospholipid antibodies to counteract this thrombosis-favoring association.

Churg-Strauss syndrome cardiac involvement evaluated by cardiac magnetic resonance imaging and positron-emission tomography: a prospective study on 20 patients

Churg-Strauss syndrome (CSS) cardiac involvement is associated with a poor prognosis. Recently cardiac MRI (CMRI) has emerged as a promising technique to detect early CSS cardiac involvement. However, CMRI-detected myocardial delayed enhancement (MDE) could correspond to fibrosis or inflammation. Fluoro-2-deoxyglucose PET (FDG-PET) was previously used in other systemic diseases to distinguish between them. To determine whether the CMRI-MDE detected in CSS patients reflected fibrosis or myocardial inflammation, patients in CSS remission underwent FDG-PET. Twenty consecutive CSS patients in remission (BVAS = 0) were recruited. Fourteen patients [eight men, six women; mean (S.D.) age 49 (9) years; mean disease duration 3.5 (2.9) years] with CMRI-detected MDE, and six patients [four men, two women; mean (S.D.) age 44 (15) years; mean disease duration 3.5 (5.3) years] with normal CMRI underwent FDG-PET. Segments with MDE on CMRI were analysed on FDG-PET images, with myocardial FDG hypofixation defining fibrosis and hyperfixation corresponding inflammation. Among the 14 patients with MDE on CMRI, FDG-PET showed 10 had hypofixation, 2 had hyperfixation and 2 had normal scans. CSS duration at the time of CMRI was shorter for patients with myocardial inflammation than in those with fibrosis. The six patients with normal CMRI had normal FDG-PET images. For CSS patients in remission, CMRI detected subclinical active myocardial lesions and could be recommended to assess cardiac involvement. However, because CMRI-detected MDE can reflect fibrosis or inflammation, FDG-PET might help to distinguish between the two.

Increased production of IL-5 and dominant Th2-type response in airways of Churg–Strauss syndrome patients

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis associated with eosinophilia and asthma. We assessed the local immune response in airways of CSS patients with different activity of the disease. Concentration of IL-5, CCL17, CCL22 and CCL26 (ELISA) together with cell expression of T-helper-related genes (real-time PCR array) were measured in bronchoalveolar lavage fluid (BALF) sampled from 11 patients with active CSS, 11 patients with CSS in remission and 9 control subjects with bronchial asthma. In active CSS, both BALF and blood eosinophil counts were increased (P<0.01). BALF cells in active disease were characterized by an increased expression of Th2 and regulatory-type transcripts: STAT6, STAT3, GATA3, IL4, IL5 and IL10 as compared with asthmatics, and STAT5A, CCR4, FOXP3, IL4, IL5 and IL10 when compared with inactive CSS. There was significant increase in BALF concentration of IL-5 and CCL26 in exacerbation of CSS. CCR4-active chemokines were detected more frequently in active disease. We found a strong positive correlation between clinical parameters of disease activity (BVAS, eosinophilia) and expression of IL4, IL5, IL10 and STAT5A. These results indicate that as compared with asthma, active-CSS patients have much stronger local Th2 response in the airways. Airway cells may contribute to lung eosinophilia in CSS by producing IL-5 and eosinophil active chemokines.

Hypereosinophilic Syndrome, Churg-Strauss Syndrome and Parasitic Diseases: Possible Links between Eosinophilia and Thrombosis

Throughout the past decade, a possible role of eosinophils in blood coagulation and thrombosis has been suggested. We conducted a Pubmed (MEDLINE) search of case and series referring to any kind of thrombotic events described in three conditions characterised by persistent blood eosinophilia, i.e. the hypereosinophilic syndrome (HES), the Churg Strauss syndrome (CSS), and parasitic infestations from 1966 to date. One hundred and ninety-two articles were found regarding thrombotic events in HES and CSS, and 209 cases of thrombosis were extracted. One hundred and seventy- seven articles dealing with parasitic diseases and thrombosis were found, but only 15 manuscripts reporting thrombosis of unknown origin in 22 patients were selected. In HES, arterial thromboses were more frequent than in CSS (p=0.006), representing almost half of the cases (45%), while venous and mixed artero-venous thrombosis were respectively 28% and 27%. In contrast, in CSS there was a predominance of venous thrombosis (56%, p=0.006), with arterial thrombosis representing 38% of total thrombotic events, and mixed thrombosis being the least frequent (4%). The higher incidence of arterial thrombosis in HES patients can be explained by the common cardiac involvement (64% of patients). In the 22 patients with parasitoses and thrombosis, 15 had arterial thrombosis (68%) and 7 had venous thrombosis (32 %). Literature analysis shows that there are numerous reports of thrombotic events in patients with eosinophil-related disorders supporting a role for eosinophils in thrombosis. This observation raises the problem of prevention and treatment of thromboembolism particularly in HES and CSS patients.

Churg-Strauss syndrome presenting with severe cutaneous and intestinal ulcers

ejd.2012.1683 Auteur(s) : Chisako Hayami1,2 c-hayami@sis.seirei.or.jp, Naoko Ishiguro2, Yasuko Fukuya2, Yoshiki Tokura1, Makoto Kawashima2 1 Department of Dermatology, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu 431-3192, Japan 2 Department of Dermatology, Tokyo Women's Medical University, Tokyo, Japan Churg-Strauss syndrome (CSS) is a representative of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis [1]. Approximately 50% of CSS patients have [...]

12‐hydroxy‐eicosatetraenoic acid (12‐HETE): a biomarker of Churg‐Strauss syndrome

Churg-Strauss syndrome (CSS) shares similarities with asthma and hypereosinophilic syndrome (HES). Eicosanoids--important inflammatory and signaling molecules--are present in exhaled breath condensate (EBC) and broncho-alveolar lavage fluid (BALF). To assess eicosanoid profile both in EBC and BALF of CSS subjects searching for a pattern characteristic of this syndrome. EBCs from 23 CSS patients, 30 asthmatics, 12 HES patients and 54 healthy controls (HC) were assessed quantitatively for 19 eicosanoids by a high-performance liquid chromatography - tandem mass spectrometry (HPLC-MS/MS). In addition, in 21 of 23 CSS subjects and in nine asthmatics, eicosanoids were determined in BALF. EBC from CSS patients showed markedly elevated levels of 12-HETE as compared with other studied groups. BALF was characterized by a significant elevation of 12-HETE and its metabolite 12-tetranor HETE in CSS as compared with asthma. Clinical activity of CSS correlated with 12-HETE and its metabolites levels in BALF, but not in EBC. CSS is clearly distinguished from bronchial asthma, and HES by a marked increase in 12-HETE concentration in both EBC and BALF. This points to a possible new pathogenic mechanism in CSS and may help in future in establishing the diagnosis of CSS.

The Mechanics of Left Ventricular Dysfunction in Patients with Churg-Strauss Syndrome

Heart is frequently involved in Churg-Strauss syndrome (CSS). However, the mechanics of left ventricular (LV) dysfunction in CSS has not been studied. To assess the mechanics of LV function and to characterize the contribution of longitudinal, circumferential and rotational deformation to LV dysfunction in CSS. We enrolled 22 CSS patients (eight males, mean age 43.2 ± 9.5 years) in remission of their disease and 22 sex- and age-matched healthy subjects. All patients underwent conventional and two-dimensional speckle-tracking echocardiography. Global longitudinal, circumferential and rotational deformation parameters were calculated. CSS subjects demonstrated lower LV ejection fraction (EF) than controls (56.6 ± 15.0% vs 63.8 ± 3.4%; P < 0.05). When compared to those with LVEF ≥ 50% (n = 14), CSS patients with LVEF < 50% (n = 7) had decreased global peak-systolic longitudinal and circumferential strain/strain rate (all P < 0.001) and tended to have lower global peak-systolic radial strain (P = 0.05). There were no differences between these two subgroups in global peak-systolic radial strain rate and LV twist/torsion. When comparing individual systolic and diastolic parameters early diastolic longitudinal and circumferential strain rate demonstrated the highest correlation with corresponding global longitudinal and circumferential peak-systolic strain/strain rate (r < -0.80, P < 0.001 for all correlations). In CSS LV systolic dysfunction strongly correlates with longitudinal and circumferential, but not radial or rotational systolic components, indicating that impaired LV systolic function may result predominantly from impaired contraction of inner and middle, but not outer myocardial fiber layers. The spatial correspondence between systolic and diastolic deformation parameters suggests the similar impact of pathologic process on systolic and diastolic function in CSS.

Churg-Strauss Syndrome Presenting with Acute Renal Insufficiency Accompanied by Eosinophilic Tubulointerstitial Nephritis

We encountered an unusual and rare case of 59-year-old woman with Churg-Strauss syndrome (CSS) showing myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)-related acute renal insufficiency accompanied by eosinophilic tubulointerstitial nephritis. To date, reports in English of CSS presenting with rapidly progressive/acute renal insufficiency and biopsy-proven renal lesions have been uncommon. Here, we discuss this unusual case and review the previously reported CSS cases. The complication of eosinophilic tubulointerstitial nephritis in CSS cases with acute renal insufficiency might be higher than generally thought. Furthermore, the presence of eosinophilic infiltration and eosinophilic tubulointerstitial nephritis might be associated with the good renal outcome in CSS patients.