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Diseases of Blood vessels
Published in P. Chopra, R. Ray, A. Saxena, Illustrated Textbook of Cardiovascular Pathology, 2013
Churg-Strauss syndrome also known as allergic granulomatous angiitis is characterized by systemic vasculitis affecting small to medium sized vessels, asthma and hypereosinophilia. Microscopically, necrotizing vasculitis with a prominent infiltrate by eosinophils is seen (Fig. 13.31). Granulomas including giant cells may also be observed. p-ANCA has been detected in several cases (about 70% cases).
Peripheral Neuropathies
Published in John W. Scadding, Nicholas A. Losseff, Clinical Neurology, 2011
Gareth Llewelyn, Robert Powell
Churg–Strauss syndrome usually causes asthma, pulmonary infiltrates, fever and eosinophilia. Neuropathy is common, occurring in 65–80 per cent of patients. ANCA is positive in around 50 per cent of patients.
Pathology of drug-induced respiratory disease
Published in Philippe Camus, Edward C Rosenow, Drug-induced and Iatrogenic Respiratory Disease, 2010
William D Travis, Douglas B Flieder
Vasculitis is a rare complication of drug toxicity.37–39 Mediumsized vessel vasculitis seen in Wegener’s granulomatosis and Churg–Strauss syndrome is not usually associated with drug reactions, although one recent case points to a WG-like syndrome developing in an ulcerative colitis patient who was taking mesalazine.40 Churg–Strauss syndrome has developed also in patients treated with leukotriene antagonists.41,42
Eosinophilic granulomatosis with polyangiitis
Published in Postgraduate Medicine, 2023
Eosinophilic granulomatosis with polyangiitis, previously known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis that affects small to medium size vessels. Jacob Churg and Lotte Strauss first identified the disease in a case series of 13 patients in 1951, describing a clinical syndrome of severe asthma, fever, and hypereosinophilia, with granulomatous lesions found in the vessels walls and extravascular tissues, including skin, lungs, and heart, and named the disease ‘allergic granulomatosis and angiitis’ [1]. Eosinophilic granulomatosis with polyangiitis is classified as part of a group of vasculitides known as ANCA-associated vasculitis [2,3]. However, the AAV encompasses a heterogeneous group of vasculitides with EGPA having very distinct clinical and histologic features. Interestingly, only 30% to 40% of patients with EGPA have a positive ANCA test [4,5]. Eosinophilic granulomatosis with polyangiitis is the rarest of all AAV with an annual incidence of approximately 0.6/million to 4/million and a prevalence of 2/million to 15/million, depending on disease definition and population. It primarily affects people between 40 and 60 years old with a mean age at diagnosis of 48 [6–11]. In a large study including 354 patients with EGPA, the male/female ratio was 41%/59%, 87.3% of patients were white, 5.6% were Asian and 2% were Black or African American [12]. Interest in EGPA as a research subject has been increasing but its rarity presents a major obstacle to the conduction of clinical trials.
Bilateral Conjunctival Granulomas in a Young Lady with Hypereosinophilic Syndrome - A Case Report
Published in Ocular Immunology and Inflammation, 2019
Kalpana Babu, Bhagya Sudheer, S. Krishna Kumar
Conjunctival granulomas have been described in sarcoidosis,1 tuberculosis,2 fungal and worm infections,3,4 foreign bodies,5 amyloidosis, Crohn’s disease,6 and Churg–Strauss syndrome.7 Our case had bilateral, multiple, and conjunctival granulomas infiltrated with eosinophils. In addition, she had hypereosinophilic syndrome with eosinophilia (19%), increased serum IgE levels, and absolute eosinophil count. She also had a history of asthma and hypereosinophilic enteritis. A complete blood work-up and stool analysis did not reveal any worm or parasite infestation. Conjunctival granulomas with eosinophilic infiltration have been described in Churg–Strauss syndrome. However, the absence of vasculitis on histopathology in our case ruled out the possibility of Churg–Strauss syndrome. We nevertheless report a rare presentation of bilateral conjunctival granulomas in a young lady with hypereosinophilic syndrome.
Mepolizumab for the treatment of eosinophilic granulomatosis with polyangiitis
Published in Expert Opinion on Biological Therapy, 2019
Daniel Ennis, Jason Kihyuk Lee, Christian Pagnoux
Although case descriptions of ‘atypical periarteritis nodosa’ with extensive pulmonary involvement can be found as early as 1905, Churg-Strauss syndrome (CSS) was not individualized and fully described until 1951 [1,2]. In their report, Drs. J. Churg and L. Strauss detailed the clinical and histologic features of 13 patients with a shared syndrome of severe asthma, fever, and hypereosinophilia, together with symptoms of ‘vascular embarrassment in various organ systems’. This series differentiated CSS from classical polyarteritis nodosa based on the presence of intravascular and extravascular eosinophilic infiltrates, severe fibrinoid necrosis, and granulomatous inflammation which they termed ‘allergic granulomatosis’ [2]. Due to the identification of ANCA in up to 40% of patients, CSS was classified as part of the ANCA-associated vasculitides (AAV) in the 1994 International Chapel Hill Consensus Conference (CHCC) along with granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA)[3]. CSS was renamed EGPA in the 2012 revised CHCC nomenclature, which also emphasized the importance of ANCA status; positivity or negativity reflecting important clinical and, possibly, prognostic differences [4].