Churg Strauss Syndrome

Hypereosinophilic syndrome (HES) is a heterogeneous group of disorders characterized by peripheral blood eosinophilia of 1.5 × 109 /L (1,500/μL) or greater, with evidence of end-organ damage attributable to eosinophilia (e.g., heart, liver or lung) with no other cause for the end-organ damage [1]. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disorder that may affect multiple organ systems (lungs, heart, kidneys, or the nervous system). The disorder is characterized by hypereosinophilia in the blood and tissues, inflammation of blood vessels (vasculitis), and the development of inflammatory nodular lesions called granulomatosis [2]...

Eosinophilic myocarditis (EM) is a rare, potentially life-threatening, form of inflammatory heart disease characterized by eosinophilic infiltration of the myocardium. Different diseases are involved in its etiopathogeneses, such as eosinophilic granulomatosis with polyangiitis (or Churg-Strauss Syndrome), hypereosinophilic syndromes, parasitic infections, drug reactions, paraneoplastic syndromes and primary immunodeficiencies (e.g. Omenn Syndrome). There is a wide spectrum of clinical pictures at presentation ranging from chronic restrictive cardiomyopathy (Loeffler cardiomyopathy) to acute necrotizing myocarditis with cardiogenic shock...

UNLABELLED: Wells syndrome or eosinophilic cellulitis is a rare and relapsing skin disease which lacks systemic involvement. A skin biopsy is needed to establish a diagnosis. Several precipitating factors have been proposed but no proven causative link has been found. On the other hand, Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), an auto-immune disease, is associated with multiorgan, including cutaneous manifestations. We report a case with overlapping features of Wells and Churg-Strauss syndrome, suggesting that these syndromes could be part of the same nosological entity...

Eosinophilic granulomatosis with polyangiitis (EGPA), historically named Churg-Strauss syndrome, is a rare vasculitis affecting small- and medium-sized blood vessels. The disease has a predilection for numerous organs including the lungs, sinuses, kidneys, heart, nerves, and gastrointestinal tract but is prominently associated with asthma, rhinosinusitis, and eosinophilia. Gastrointestinal involvement is common; however, a gastrointestinal manifestation as the cardinal symptom following an infection is atypical...

OBJECTIVES: To analyse effectiveness, safety, and steroid-sparing effect of AZA and MTX as induction of remission and maintenance treatment in eosinophilic granulomatosis with polyangiitis. METHODS: We retrospectively collected data from 57 patients divided in 4 groups according to treatment: MTX/AZA as first-line agents (MTX1/AZA1) in non-severe disease, or as second-line maintenance therapy (MTX2/AZA2) in severe disease previously treated with CYC/rituximab. During the first five years of treatment with AZA/MTX we compared the groups according to: remission rate (defined as R1: BVAS = 0, R2: BVAS = 0 with prednisone ≤5mg/day, R3-MIRRA definition: BVAS = 0 with prednisone ≤ 3...

Eosinophilic granulomatosis with polyangiitis (EGPA) is an uncommon antineutrophil cytoplasmatic antibody (ANCA) associated vasculitis involving small and medium size blood vessels. It has a variable clinical presentation depending on the main organ involved, making it difficult to diagnose. Treatment is mainly based on high-dose steroids and other immunosuppressants like cyclophosphamide, which may prevent end-organ damage and induce remission at the expense of having important adverse effects. However, new therapeutic agents had been shown to provide better results with favorable safety profiles...

BACKGROUND: The mainstay of treatment for eosinophilic granulomatosis with polyangiitis (EGPA) is systemic corticosteroid therapy; some patients also receive intravenous immunoglobulins, other immunosuppressive agents, and biologics. Mepolizumab, an anti-interleukin-5 monoclonal antibody, induces remission and decreases the daily corticosteroid dose; however, the clinical efficacy of mepolizumab in EGPA and the prognosis with long-term treatment with this drug are unknown. METHODS: Seventy-one EGPA patients were treated at Hiratsuka City Hospital, Japan, between April 2018 and March 2022...

We present a case of a 47-year-old woman with a history of asthma and mononeuritis who presented with shortness of breath and fatigue. Heart failure was diagnosed and echocardiography revealed large floating thrombi attached to the left ventricular walls. Cardiac magnetic resonance imaging showed evidence of myocarditis and angiitis. Blood count revealed eosinophilia. She was diagnosed with eosinophilic granulomatosis with polyangiitis or Churg-Strauss syndrome (CSS) according to recently updated criteria. Medical management with specific aetiology (anticoagulation or immunosuppression) and heart failure treatment resulted in clinical improvement...

CASE PRESENTATION: A 63-year-old man presented with fever, thoracalgia, weight loss, diffuse lymphadenopathy, and a massive pleural effusion. Extensive laboratory and radiologic investigations for possible autoimmune, infectious, hematologic, and neoplastic conditions all resulted negative. A lymph node biopsy showed a granulomatous necrotizing lymphadenitis, suspicious for tuberculosis. Although mycobacterium tuberculosis (MT) was never isolated and tuberculin skin test resulted negative, diagnosis of extrapulmonary tuberculosis was made and anti-tubercular therapy was started...

Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a multisystem disorder characterised by asthma, blood and tissue eosinophilia and small-vessel vasculitis. Eosinophilic tissue infiltration and extravascular granuloma formation can lead to damage in any organ, but it is classically seen to cause pulmonary infiltrates, sino-nasal disease, peripheral neuropathy, renal and cardiac involvement, and rashes. EGPA is part of the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndromes, with the antibody being detected in ∼30-40% of cases and mostly against myeloperoxidase...

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of systemic disease characterized by inflammation and necrotizing effects of the small and medium blood vessels. It is a vasculitis found in all age groups and both genders, although its etiology is unknown. The mean age at diagnosis is 40 years, consisting of an uncommon cause of vasculitis in people older than 65 years. It is the least common of the three antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (EGPA, granulomatosis with polyangiitis (GPA), and microscopic polyangiitis)...

INTRODUCTION: Eosinophilic cystitis is a rare condition which causes common symptoms and may mimic other conditions. Eosinophilic cystitis has several causes such as hypereosinophilic syndrome, inflammatory diseases, neoplasia, parasites or fungal infection, IgE-related diseases, Drug Reaction and Eosinophilia and Systemic Symptoms (DRESS) syndrome, or Churg-Strauss syndrome. Therefore, differential diagnosis is difficult. CASE PRESENTATION: We report the case of a middle-aged man affected by eosinophilic cystitis with persistent hematuria and other peculiar symptoms that may be brought back to hypereosinophilic crisis...

Eosinophilic granulomatosis with polyangiitis is a systemic vasculitis characterized by the presence of asthma, hyper-eosinophilia, and necrotizing vasculitis with extravascular eosinophilic granulomas. We report the case of a 25-year-old male who presented to the outpatient department complaining of joint aches and numbness in the hands and legs. Physical examination revealed erythematous blanchable macular rashes on palms and soles. Raynaud's phenomenon was also observed. Lab workup revealed elevated WBC count and peripheral blood eosinophilia...

Churg-Strauss syndrome is a rare disease with systemic vasculitis and hypereosinophilia. It is accompanied by allergic rhinitis and asthma. Herein, we present a case of Churg-Strauss syndrome in a family medicine outpatient clinic. This report aimed to emphasize that if patients diagnosed with asthma have cough and/or hemoptysis that cannot be controlled despite interventions such as inhalation, corticosteroid therapy, montelukast treatment, and anti-histamine therapy, vasculitic diseases involving the lung may be considered...

Eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) is classified as an anti-neutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis. It is a multisystem disorder and can affect every organ system. EGPA is a rare disease, with an estimated prevalence of 1/70,000-100,000 in Europe. As its onset usually occurs in adulthood, data from paediatric patients are limited. We present here a very rare practical EGPA clinical case involving a paediatric patient...

Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a rare reaction pattern that is frequently linked to several systemic diseases, including autoimmune disease, inflammatory bowel disease, and vasculitis. Churg-Strauss syndrome (CSS) is an uncommon systemic condition that occurs exclusively in patients with asthma or a history of atopy. It is characterized by extravascular necrotizing granuloma and hypereosinophilia. This case report describes an illustrative case of a 61-year-old Bahraini female who had been diagnosed with CSS and presented with PNGD...

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, multi-system, inflammatory disease, belonging to the group of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). Previously known as Churg-Strauss syndrome, EGPA is characterised by late-onset asthma, eosinophilia and vasculitis affecting small-to-medium vessels. This disease behaves differently in many aspects to the other AAV and is often excluded from AAV studies. The disease is poorly understood and, due to it rarity and unique manifestations, there has been limited research progress to optimise our understanding of its complex pathogenesis and ability to develop management options - although the success of interleukin-5 inhibitors such as Mepolizumab has been a welcome development...

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem disorder; cardiac involvement may include eosinophilic myocarditis. A 67-year-old woman presented with 1-week history of dyspnoea and orthopnoea. She had a history of adult-onset asthma and peripheral eosinophilia. The investigations showed T-wave inversion on lateral leads, peripheral eosinophilia, elevated troponin and BNP values, and severe biventricular systolic dysfunction with diffuse hypokinesia and apical akinesia. Computed tomography excluded coronary disease and showed bilateral basal ground-glass opacities, air-space consolidation, and bilateral reticular-nodular pattern...

Torasemide is a loop diuretic with a molecule that is chemically similar to the sulphonamides described as eosinophilic granulomatosis with polyangiitis (EGPA) triggering drugs. The presented case is probably the first description of torasemide-induced vascular purpura in the course of EGPA. Any diagnosis of vasculitis should be followed by an identification of drugs that may aggravate the disease. A 74-year-old patient was admitted to the Department of Dermatology with purpura-like skin lesions on the upper, and lower extremities, including the buttocks...

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by the presence of asthma and eosinophilia. Because cerebral aneurysm formation induced by EGPA is a rare occurrence, there is no established treatment strategy for this condition. OBSERVATIONS: A 67-year-old female who was diagnosed with idiopathic eosinophilia 3 months ago developed de novo fusiform aneurysms in the left vertebral, left internal carotid, and bilateral superficial temporal arteries, as noted during a regular follow-up examination of a convexity meningioma...