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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.
Pulmonary Eosinophilia
Published in Philip T. Cagle, Timothy C. Allen, Mary Beth Beasley, Diagnostic Pulmonary Pathology, 2008
The pathogenesis of Churg-Strauss syndrome is not well understood. Original studies postulated a role for immune complexes based on increased serum IgE levels and immune complexes containing IgE. More recent studies have noted the presence of ANCA in a significant minority (38%) of patients most of which are antimyeloperoxidase. Based on these findings, some authors have suggested that Churg-Strauss syndrome is heterogeneous with distinct clinical phenotypes (ANCA-positive and -negative) reflecting different underlying pathogenetic mechanisms. This suggestion is bolstered by the observation that the clinical manifestations of ANCA-positive patients tend to be dominated by vasculitic complications (alveolar hemorrhage, necrotizing glomerulonephritis, purpura, and/or mononeuritis multiplex) while those of ANCA-negative patients are dominated by the effects of eosinophilic tissue infiltration (eosinophilic pneumonia, cardiomyopathy, mono-/ polyneuropathy, eosinophilic gastritis/enteritis) (142,143).
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].
Step-up and step-down treatments for optimal asthma control in children and adolescents
Published in Journal of Asthma, 2019
Jonathan A. Bernstein, Lyndon Mansfield
LTRAs have the advantage of being administered orally once or twice daily. And importantly, unlike long-term ICS use, they do not adversely affect growth or the adrenal axis [37]. However, LTRAs have been associated with other adverse drug reactions. For example, montelukast-induced adverse drug reactions include psychiatric and nervous system disorders like agitation, anxiety, depression, sleep disturbance, hallucinations, suicidal thinking and suicidality, tremor, dizziness, drowsiness, neuropathies, and seizures. Immune system involvement may lead to Churg-Strauss syndrome. Hypersensitivity reactions like anaphylaxis and eosinophilic infiltration may also occur with LTRA use [38].