Strauss Syndrome Research Articles

Two cases of atypical hemolytic uremic syndrome (aHUS) and eosinophilic granulomatosis with polyangiitis (EGPA): a possible relationship.

Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by hemolysis, thrombocytopenia, and renal failure. It is related to genetic mutations of the alternative complement pathway and is difficult to differentiate from other prothrombotic microangiopathies. Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss syndrome, CSS) is a systemic ANCA-associated vasculitis and a hypereosinophilic disorder where eosinophils seem to induce cell apoptosis and necrosis and therefore, vasculitis. Here, we report the case of two CSS patients with a genetic complement disorder consistent with aHUS diagnosis. Both patients showed histologic features that supported the diagnosis of CSS, and a genetic complement study confirmed the suspected aHUS diagnosis. In the case where eculizumab was administered, the global response was excellent. There is very limited understanding of the genetics and epidemiology of both, atypical HUS and EGPA, but considering our two patients we suggest that an etiopathogenic link exists among patients diagnosed with both entities.

Pulmonary vasculitis and pulmonary hemorrhage

Pulmonary vasculitis is a rare disease that typically shows inflammation in pulmonary vessel walls and necrosis.1 The disease is usually immune mediated, common in young patients, triggered by many factors, and with wide clinical and radiologic presentations. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAVs) is the main focus in most literature and includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), Churg–Strauss syndrome (CSS), idiopathic pauci immune pulmonary capillaritis (IPIPC), secondary to systemic lupus erythematosus, and other types like anti-glomerular basement membrane disease, drug-induced vasculitis after cannabis and glue inhalation.2 Diagnosis is usually established after careful history taking clinical, radiologic, and laboratory evidence; bilateral lung infiltrates with or without pulmonary hemorrhages and peripheral distribution, positive ANCA-P component, proteinase-3 ELISA test, anti-glomerular basement membrane antibody, and other antibodies associated with immunologic disease like systemic lupus erythematosus. Sometimes, lung biopsy may be needed but because of the low yield of bronchoscopic biopsies and the need for open lung biopsy, this tool is rarely used specially in critically ill patients. Treatment is by the use of steroids and cytotoxic medications like cyclophosphamide and mycophenolate mofetil. In refractory cases, plasmapheresis or the monoclonal antibody rituximab may be used. Pulmonary hemorrhage is a common manifestation in vasculitic lung syndromes; however, many other disease and toxins can trigger pulmonary hemorrhage, which can be life threatening.3 The diagnosis of pulmonary hemorrhage is generally considered based on clinical and radiological findings and is characterized by acute bilateral patchy lung infiltrates more centrally located but may be diffuse. Sequential aliquots of bronchoalveolar lavage yield progressively bloodier return and help in diagnosis.4 Treatment depends largely on the cause. Extracorporeal membrane oxygenation (ECMO) is a life support modality that can be used in severe cases of pulmonary vasculitis and hemorrhage with great caution and careful assessment of risk of bleeding with anticoagulation and benefit of use as life-saving support. With newer ECMO circuits used (either heparin- or bioline-coated circuits), several days of heparin-free ECMO runs may be successful. In some reported cases, citrate was used as an anticoagulant.5 The ECMO modality shall be carefully chosen especially in the presence of pulmonary hypertension, which may affect the veno-venous (VV) ECMO flow, and hence, echocardiographic assessment before initiation is mandatory to avoid low-efficiency runs. In cases with severe pulmonary hypertension, veno-arterial (VA) ECMO would be more suitable. Institution of treatment using steroids and cytotoxic drugs in addition to plasmapheresis is warranted upon suspected diagnosis. The rapid initiation of treatment may be the key point of survival in such cases. There are some case reports where plasmapheresis and immunosuppressive therapies were used based on clinical and radiographic data alone5 with successful outcome. There is paucity of data regarding drug doses of immunosuppressive drugs used during the ECMO run, and the duration of treatment was variable. Most of the cases reported started with 1 g of methylprednisolone for 3 days followed by either plasmapheresis and cyclophosphamide or cyclophosphamide and rituximab.6 The plasmapheresis circuit installation needs to be addressed whether to be incorporated into the ECMO circuit7 or not.

Cardiovascular magnetic resonance imaging pattern at the time of diagnosis of treatment naïve patients with connective tissue diseases

Background-aimCardiac involvement at diagnosis of connective tissue disease (CTD) has been described by echocardiography. We hypothesized that cardio-vascular magnetic resonance (CMR) detects occult lesions at CTD diagnosis. Patients-methodsCMR was performed early after diagnosis in 78 treatment-naïve CTDs (aged 43±11, 59F/19M) without cardiac involvement [5 Takayasu arteritis (TA), 4 Churg Strauss syndrome (CSS), 5 Wegener granulomatosis (WG), 16 systemic lupus erythematosus (SLE), 12 rheumatoid arthritis (RA), 8 mixed connective tissue diseases (MCTD), 12 ankylosing spondylitis (AS), 3 polymyalgia rheumatica (PMR), 8 systemic sclerosis (SSc) and 5 dermatomyositis (DM)]. Acute and chronic lesions were assessed by T2>2 with positive LGE and T2<2 with positive LGE, respectively. ResultsIn 3/5 TA, 3/4 CSS, 4/5 WG, 10/16 SLE, 9/12 RA, 6/8 MCTD, 4/12 AS, 1/3 PMR, 2/8 SSc and 2/5 DM, the T2 ratio was higher compared to normal (2.78±0.25 vs 1.5±0.2, p<0.01). Myocarditis was identified in 1 TA, 1 SLE, 1 RA, 1 SSc and 2 DM patients; diffuse, subendocardial fibrosis in 1 CSS and 1 RA patient, while subendocardial myocardial infarction in 3 SLE, 1 MCTD, 1 PMR and 2 RA patients. CMR re-evaluation after 6 and 12months of rheumatic and cardiac treatment, available in 28/52 CTDs with increased T2 ratio, showed significant improvement in T2 ratio (p<0.001), non-significant change in LGE extent and normalisation of those with impaired LV function. ConclusionsOccult CMR lesions, including oedema, myocarditis, diffuse subendocardial fibrosis and myocardial infarction are not unusual in treatment naïve CTDs and may be reversed with appropriate treatment.

CT manifestations of Churge⁃Strauss syndrome: a case report

CT manifestations of Churge⁃Strauss syndrome: a case report

Coronary Artery Vasospasm in a Patient with Probable Churg Strauss Syndrome

Case: A 56-year-old male ex-smoker presented with an inferoposterior STEMI, after recent episodes of Prinzmetal angina. His history included dyslipidaemia, adult-onset asthma, allergic rhinitis, and gastric reflux. Whilst awaiting primary PCI, the patient suffered a bradycardic arrest, necessitating CPR, thrombolysis and DC cardioversion for VF, resulting in ROSC (total resuscitation time 40 minutes). Coronary angiography revealed only minor coronary artery disease. Findings were in keeping with coronary artery vasospasm, and a calcium channel blocker and nitrate were commenced. An AICD was inserted. Peripheral eosinophilia (4.0 × 109/L) was present, and further investigation revealed that the patient had eosinophilic infiltrates in the sinuses and oesophagus, an elevated IgE and a positive ANCA. Respiratory function tests revealed a moderately severe obstructive defect. CT imaging showed extensive opacification of the sinuses, nasal polyposis and diffuse oesophageal thickening. A diagnosis of probable Churg Strauss Syndrome (CSS) was made, although definitive evidence of vasculitis was lacking. Other hypereosinophilic disorders were excluded or thought unlikely. The patient was commenced on prednisolone and azathioprine, with almost complete control of asthma, rhinitis and oesophageal symptoms, associated with normalisation of the eosinophil count. He has been free of further cardiac events for over 12 months. Conclusion: Although CSS is associated with a number of cardiac manifestations, including myocarditis, heart failure, and sudden death, reports of coronary vasospasm are rare. This is a rare case of a patient with vasospastic acute coronary syndrome, who was eventually diagnosed with probable CSS. Patients presenting with cardiac pathology and eosinophilia should have further investigations.

ПОРАЖЕНИЕ СЕРДЦА ПРИ СИНДРОМЕ ЧАРДЖА–СТРОСС

Cardiac involvement is the most important prognostic factor in eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome). We report a case of Churg–Strauss syndrome in 65-year-old women masquerading as a non-ST elevation myocardial infarction. She had chest discomfort, dyspnea and ST depression, high troponin level and so myocardial infarction was diagnosed. She had had asthma for 4 years but had no eosinophilia in peripheral blood and lesions in the lungs at the time of the first hospitalization. Her skin was clean without rashes. 3 months later she was hospitalized again having pulmonary infiltrates. Laboratory tests revealed that eosinophil was significantly increased. Cardiac involvement in a pathological process led to death. Histological examination of heart and lungs showed necrotic coronary vasculitis, granulomas and perivascular eosinophilic infiltrates in myocardium, endocardium, pericardium and pulmonary eosinophilic infiltrates.

Churg–Strauss syndrome associated with antiphospholipid antibodies in a patient with retinal vasculitis

Abstract Case report We present the case of a 69-year-old woman with unilateral retinal vasculitis. Investigations showed asthma, rhinosinusitis, nasal polyposis, peripheral blood eosinophilia, increased sedimentation rate, proteinuria, and antiphospholipid antibodies. Anti-neutrophil cytoplasmic antibodies (ANCA) were negative. Discussion Although her anti-neutrophil cytoplasmatic antibody (ANCA) status was negative, taking into account the other clinical and laboratory features, retinal vasculitis was thought to be an ocular manifestation of Churg–Strauss syndrome. Treatment was started with high-dose corticosteroids and anticoagulant therapy.

Not All Coughs Are Asthma or Allergies: Churg–Strauss Syndrome

BackgroundCardiomyopathy in patients with Churg–Strauss syndrome (CSS) carries a poor prognosis, with a high 5-year mortality rate, and requires treatment with immunosuppressive therapy. There is no single pathognomonic test or clinical finding for diagnosing CSS; instead, it is based upon meeting four of six criteria. Case ReportA 47-year-old woman with a 6-month medical history of “asthma” presented to our hospital with a 1-month history or dyspnea on exertion. She denied any chest pain, cough, fever, orthopnea, or leg swelling. She was afebrile and normotensive, and her physical examination was unremarkable. Her white blood cell count was 23,000/mm3 with 23% eosinophils, and her troponin T level was 1.08 ​Ng/ml. Extensive work-up revealed CSS. Why Should an Emergency Physician Be Aware of This?Emergency physicians should be aware of unusual cases because not every patient that walks into an emergency department has a simple case of “asthma” or “allergies.” Our patient had been diagnosed with asthma for 6 months before her symptoms had progressed to a point that prompted her to seek medical attention in a hospital. Emergency physicians are in a unique position to identify patients who present with recurrent complaints of asthma—especially late-onset asthma, which gradually worsens and is refractory to usual treatment. A complete blood cell count with a differential will prove valuable. Assessments of a patient's C-reactive protein level and erythrocyte sedimentation rate are inexpensive and check for signs of inflammation, although they are nonspecific. A chest radiograph or computed tomography scan of the chest or sinuses in some patients can also prove to be of value. Prompt recognition and treatment with steroids is imperative to ablate vasculitis tissue damage because this can improve the outcome.

Compression in vasculitis

Not available

Churg-Strauss syndrome in a pediatric patient presenting with recurrent hemorrhagic stroke and mitral regurgitation due to a prolapsed mitral valve.

Churg-Strauss syndrome in a pediatric patient presenting with recurrent hemorrhagic stroke and mitral regurgitation due to a prolapsed mitral valve.

Association Between Churg Strauss Syndrome and Vitamin D Deficiency: A Myth or Truth? A Rare Case Report

Churg-Strauss syndrome is a vasculitis of medium to small sized vessels. Diagnosis is mainly clinical with findings of asthma, eosinophilia, rhinosinusitis and signs of vasculitis in major organs. Vitamin D is thought to be important for maintaining normal function of many non-skeletal tissues such as muscle (including heart muscle), immune function, and inflammation as well as cell proliferation and differentiation. The authors are reporting a case of 56 yrs. old male, a known patient of reactive airway disease who was diagnosed to have Churg Strauss Syndrome associated with severe Vitamin D deficiency and degenerative bone disease. There is a deficiency of adequate documentation of this syndrome along with Vitamin D deficiency in the present medical literature available.

79. Heart transplantation in a patient with eosinophilic granulomatosis with polyangitis (Churg–Strauss syndrome)

Background Churg–Strauss syndrome, recently renamed eosinophilic granulomatosis with polyangitis (EGPA), was first described in 1951. It is characterized by blood and tissue eosinophilia and disseminated necrotizing vasculitis in asthmatic patients (1). Heart involvement is the leading cause of death in most of these patients which occurs in approximately 8–20% of EGPA patients and is more frequent in ANCA −-ve patients (2). Case We report a 32 years old man with Churg–Strauss syndrome. He presented with a history of bronchial asthma and corticosteroid treatment. The patient developed severe heart failure necessitating heart transplantation in May 2014. His post operative course was uneventful except for tricuspid regurgitation which subsided during the first two months follow-up. Up till now, the patient has had 5 endomyocardial biopsies with no evidence of acute rejection (grade 0) or recurrence of EGPA. Conclusion Only 9 patients with EGPA (Churg–Strauss syndrome) who received heart transplantation have been reported in a retrospective international multicentre study (3). To our knowledge this is the 10th case of heart transplantation in a patient with EGPA (Churg–Strauss syndrome).

PP.27.19

Objective: The heart is frequently affected in systemic autoimmune diseases. Any part of the heart can be affected, including the pericardium, myocardium, valves, and the conduction system. The aim of our study is to outline the cardiac manifestations of the systemic autoimmune diseases. Design and method: This retrospective study concerned cases of cardiac involvement due to systemic autoimmune diseases; followed by internal diseases department between 2000 and 2015. Results: They were 26 cases with 6 men and 20 women. The mean age at onset of cardiac involvement was 32 years. The most common symptoms were progressive dyspnea and chest pain occurring in respectively 50% and 20% of cases. Asymptomatic heart involvement occurred in 30% of cases. Pericarditis was the main heart manifestation occurring in 77% of cases. Myocarditis was observed in all the other cases. The causes of pericarditis included: Systemic Lupus Erythematosus (65%), Systemic Sclerosis (8%), lupus scleroderma overlap, mixed connective tissue disease and adult still's disease in respectively one case. Vasculitis complicated with pericarditis was seen in two cases: takayashu vasculitis and Churg Strauss syndrome. The etiologies of myocarditis were: Systemic Lupus Erythematosus (3 cases), Churg Strauss syndrome (1 case) and Kawasaki disease in a sixteen years old patient. The treatment was based on corticoids in all cases. Immunosuppressive treatment with cyclophosphamide was indicated in seven cases. The response to therapy was favorable in 92% of cases. Conclusions: Cardiac involvement in patients with systemic autoimmune diseases is common. Improved technology has made the diagnosis of many of these conditions easier. The prognosis is generally conserved.

Eosinophilic granulomatosis with polyangiitis (Churg–Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management

ObjectiveTo develop disease-specific recommendations for the diagnosis and management of eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) (EGPA). MethodsThe EGPA Consensus Task Force experts comprised 8 pulmonologists, 6 internists, 4 rheumatologists, 3 nephrologists, 1 pathologist and 1 allergist from 5 European countries and the USA. Using a modified Delphi process, a list of 40 questions was elaborated by 2 members and sent to all participants prior to the meeting. Concurrently, an extensive literature search was undertaken with publications assigned with a level of evidence according to accepted criteria. Drafts of the recommendations were circulated for review to all members until final consensus was reached. ResultsTwenty-two recommendations concerning the diagnosis, initial evaluation, treatment and monitoring of EGPA patients were established. The relevant published information on EGPA, antineutrophil-cytoplasm antibody-associated vasculitides, hypereosinophilic syndromes and eosinophilic asthma supporting these recommendations was also reviewed. DiscussionThese recommendations aim to give physicians tools for effective and individual management of EGPA patients, and to provide guidance for further targeted research.

Autoantibodies in systemic vasculitis.

Autoantibodies in systemic vasculitis.

Churg–Strauss Syndrome Presenting with Endobronchial Masses

Churg–Strauss syndrome is a condition with unknown etiology and asthma, allergic rhinitis, eosinophilic infiltration of blood and tissues, and transient infiltration of the lungs. It occurs mostly in the 3rd–4th decades of life with an incidence of 2.4/1000000. Presentation frequently involves nodular lung infiltrations, infiltrations with cavity, ground-glass appearance, and alveolar opacity. However, endobronchial mass is an unexpected presentation. In the current case report, we present a 45-year-old male patient who was receiving asthma therapy for 5 years. In the last follow-up visit, we identified a mass in the right hilum on X-ray radiography and performed fiberoptic bronchoscopy. Pathologic examination of biopsy material verified the diagnosis of Churg–Strauss syndrome. Bronchial mass is an unexpected presentation of Churg–Strauss syndrome and pathologic examination is essential to distinguish it from pulmonary malignancies

Intravenous immunoglobulin for chronic residual peripheral neuropathy in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): a multicenter, double-blind trial.

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg–Strauss syndrome, frequently affects the peripheral nervous system. We conducted a multicenter, double-blind, three-arm treatment period, randomized, pre-post trial to assess the efficacy of intravenous immunoglobulin (IVIg) administration for residual peripheral neuropathy in patients with EGPA that is in remission, indicated by laboratory indices. Twenty-three patients were randomly assigned into three groups, in which the timing of IVIg and placebo administration was different. Each group received one course of intervention and two courses of placebo at 2-week intervals. Treatment effects were assessed every 2 weeks for 8 weeks. The primary outcome measure, the amount of change in the manual muscle testing sum score 2 weeks after IVIg administration, significantly increased (p = 0.002). The results over time suggested that this effect continued until the last assessment was done 8 weeks later. The number of muscles with manual muscle testing scores of three or less (p = 0.004) and the neuropathic pain scores represented by the visual analogue scale (p = 0.005) also improved significantly 2 weeks after IVIg administration. This study indicates that IVIg treatment for EGPA patients with residual peripheral neuropathy should be considered even when laboratory indices suggest remission of the disease.

Efficiency of using rituximab in a patient with generalized granulomatosis with polyangiitis: A case report

Systemic vasculitides (SVs) are characterized by inflammation of the blood vessels wall; the spectrum of their clinical manifestations depends on the type, extent, and location of affected vessels and the activity of systemic inflammation. The etiology of most primary SVs is unknown. Antineutrophil cytoplasmic antibodies (ANCAs) are implicated in its pathogenesis. The presence of ANCAa in patients' serum and the correlation of their level with the severity of clinical manifestations served as a basis for identifying a subgroup of systemic necrotizing vasculitides associated with ANCA synthesis: granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and Churg – Strauss syndrome. GPA is characterized by systemic granulomatous necrotizing vasculitis involving the small vessels of the upper respiratory tract, lung, and kidney. The paper describes a case of difficult diagnosis and successful rituximab (RTM) treatment of generalized GPA in a 45-year-old female patients. The disease occurred with local damage to the upper respiratory tract, granulomatous inflammation of the pulmonary vessels to form multiple infiltrates with lung tissue destruction elements and necrotizing glomerulonephritis. Despite intensive immunosuppressive treatment, there was a rapid GPA progression with the further development of respiratory failure, which had been induced by stenotic laryngitis subglottica leading to tracheostoma. Damage to the organ of vision could lead to severe complications, including amaurosis. RMT was shown to be effective in treating generalized GPA with a poor prognosis.

Takayasu's arteritis- experience in a tertiary care centre in eastern India

Introduction: Limited data on Churg Strauss syndrome from western India. Aims: To describe the clinical profile and outcome of patients with Churg Strauss syndrome. Methods: Demographic profile, clinical, laboratory and treatment details, BVAS, EULARdisease stages and therapeutic outcome of 28 patients presented at two centers from 2003-2014 were analyzed. Results: There were 28 patients [16 (57.14%) males & 12 (42.86%) females]. Mean age at presentation was 46.6±15.14 yrs and mean duration of illness at diagnosis was 18.1±17.2 months. Mean duration of asthma prior to CSS was 4.4 ± 5.6 years. The most common systemic features were peripheral neuropathy 25 (89.28%) and pulmonary infiltrate 12 (42.85%). ANCA was positive in 22/28 (78.57%) patients. Mean BVAS at presentation was 14.7 ±5.6. The EULAR disease stage at presentation was early systemic 25 (89.28%), and generalized 3 (10.71%). Steroid was used in induction (Parenteral 25%, oral 75%) and maintenance in all patients. Other immunosuppressant's used for induction were cyclophosphamide 22 (78.57%), azathioprine 5 (17.85%) and methotrexate 1 (3.57%). Maintenance immunosuppressant's were azathioprine 11 (39.28%), methotrexate 6 (21.42%), cyclophosphamide 7 (25%), only steroid 3 (10.71%) and Mycofenolate 1(3.57%). The mean duration of follow-up was 46.9 ± 29.3 months with remission in 27 (96.42%) patient after mean duration of 6.5 ± 2.5 months. Mean number of relapses were 1.4 ± 1.8. No death occurred during follow up. Conclusions: Apart from chest symptoms, peripheral neuropathy was an important manifestation. Steroids with cyclophosphamide were most commonly used for induction. Remission was achieved in most and relapses were uncommon.

Eosinophilic granulomatosis with polyangitis (Churg–Strauss syndrome): a diagnostic rarity with an atypical presentation

We report a case of a 33-year-old woman who presented to us with symptoms of bronchial asthma and peripheral neuropathy. After investigations, the diagnosis of eosinophilic granulomatosis and polyangitis (Churg-Strauss syndrome) was made.