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Churg-Strauss Syndrome (Allergic Angiitis)

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Widespread necrotizing angiitis with granulomas. Pulmonary involvement is frequent. Asthma or other respiratory infection may precede evidence of vasculitis. Eosinophilia and lung involvement differentiate this disease from POLYARTERITIS NODOSA.

Also Known As:

Allergic Angiitis; Syndrome, Churg-Strauss; Vasculitis, Churg Strauss; Allergic Angiitides; Allergic Granulomatous Angiitides; Angiitides, Allergic; Angiitides, Allergic Granulomatous; Angiitis, Allergic; Churg Strauss Syndrome; Churg-Strauss Vasculitis; Granulomatous Angiitides, Allergic; Granulomatous Angiitis, Allergic; Allergic Granulomatous Angiitis; Angiitis, Allergic Granulomatous; Vasculitis, Churg-Strauss

Networked: 742 relevant articles (29 outcomes, 21 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Vasculitis (Vasculitides)
2.	Wegener Granulomatosis (Wegener's Granulomatosis)
3.	Microscopic Polyangiitis
4.	Polyarteritis Nodosa (Periarteritis Nodosa)
5.	Systemic Vasculitis

Experts

1.	Guillevin, Loïc: 23 articles (08/2014 - 02/2002)
2.	Pagnoux, Christian: 16 articles (02/2014 - 04/2003)
3.	Gross, W L: 13 articles (09/2009 - 03/2000)
4.	Mouthon, Luc: 12 articles (05/2014 - 11/2003)
5.	Gross, Wolfgang L: 12 articles (05/2013 - 05/2002)
6.	Hellmich, B: 10 articles (11/2012 - 07/2003)
7.	Moosig, Frank: 9 articles (08/2014 - 06/2008)
8.	Guillevin, L: 9 articles (07/2011 - 04/2001)
9.	Csernok, Elena: 8 articles (08/2014 - 05/2002)
10.	Mahr, Alfred: 7 articles (01/2014 - 11/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Churg-Strauss Syndrome:

1.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 05/15/2007 - "To compare long and short durations of adjunctive cyclophosphamide for the treatment of severe Churg-Strauss syndrome (CSS). " 05/01/2007 - "Thus cyclophosphamide is only used in patients with generalized disease or - regarding Churg-Strauss-syndrome - patients with risk factors. " 06/01/2002 - "We present a case of a woman diagnosed with Churg-Strauss syndrome that was refractory to corticosteroid and cyclophosphamide treatment. " 01/01/2009 - "Repressed ileal artery aneurysms in Churg-Strauss syndrome following combination treatment with glucocorticoid and cyclophosphamide." 02/01/2004 - "Mononeuritis in Churg-Strauss syndrome in Asians responding to intravenous cyclophosphamide."
2.	Interferon-alpha (Interferon Alfa)FDA Link 09/01/1998 - "Interferon-alpha may be an effective treatment for the Churg-Strauss syndrome. " 01/01/2010 - "Interferon-alpha for maintenance of remission in Churg-Strauss syndrome: a long-term observational study." 01/01/2010 - "To examine the safety and efficacy of interferon-alpha for mainten-ance of remission in Churg-Strauss syndrome. " 05/01/2008 - "A phase II study of interferon-alpha for the treatment of refractory Churg-Strauss syndrome." 09/01/1998 - "To study the effect of interferon-alpha on the Churg-Strauss syndrome. "
3.	Immunoglobulins (Immunoglobulin)IBA 12/01/2004 - "Long term effectiveness of intravenous immunoglobulin in Churg-Strauss syndrome." 01/01/2004 - "Treatment of Churg-Strauss syndrome with high-dose intravenous immunoglobulin." 06/01/2002 - "Churg-Strauss syndrome and pregnancy: successful treatment with intravenous immunoglobulin." 11/01/1991 - "Treatment of Churg-Strauss syndrome with high-dose intravenous immunoglobulin." 12/01/2004 - "Complete clinical and functional recovery with a long term stable remission and a low incidence of side effects can be achieved by intravenous immunoglobulin associated with plasmapheresis in patients with Churg-Strauss syndrome."
4.	Adrenal Cortex Hormones (Corticosteroids)IBA 10/01/2009 - "Churg-Strauss syndrome secondary to antileucotriene therapy in a patient not receiving oral corticosteroids." 08/08/2007 - "In Churg-Strauss syndrome, treatment can be started with high doses of corticosteroids, tapering them when the clinical situation improves. " 03/01/2007 - "To describe an unusual case of Churg-Strauss syndrome (CSS) that presented with a conjunctival nodule and was successfully treated with oral corticosteroids. " 07/01/2004 - "The patient was finally diagnosed with Churg-Strauss syndrome and was treated with oral corticosteroids. " 06/01/2002 - "The initial management of Churg-Strauss syndrome consists of high doses of corticosteroids. "
5.	Antineutrophil Cytoplasmic Antibodies (ANCA)IBA 08/01/2014 - "Heart involvement is the leading cause of death of patients with eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome) and is more frequent in anti-neutrophil cytoplasm antibody (ANCA)-negative patients. " 10/01/2013 - "Cardiac involvement in ANCA (+) and ANCA (-) Churg-Strauss syndrome evaluated by cardiovascular magnetic resonance." 02/01/2013 - "The clinical significance of antineutrophil cytoplasmic antibodies (ANCAs) in the phenotypic expression of Churg-Strauss syndrome (CSS) is uncertain. " 01/01/2012 - "Clinical presentation of Churg-Strauss syndrome in children: A 12-year-old-boy with ANCA-negative Churg-Strauss syndrome." 01/01/2012 - "Although her antineutrophil cytoplasmic antibody (ANCA) status was negative, CRAO was thought to be an ocular manifestation of Churg-Strauss syndrome, and appropriate treatment was planned. "
6.	Prednisolone (Predate)FDA LinkGeneric 11/01/2000 - "Churg-Strauss syndrome was diagnosed and prednisolone was administered with a good response leading to remission. " 01/01/2012 - "At the age of 46, a Japanese woman developed Churg-Strauss syndrome that later became stable with low-dose prednisolone treatment. " 01/01/2012 - "He received prednisolone treatment for Churg-Strauss syndrome. " 01/01/2012 - "She was diagnosed to have Churg-Strauss syndrome and had remarkable clinical improvement on day 5 with high-dose of oral prednisolone (50 mg/day). " 01/01/2011 - "Successful multidrug treatment of a pediatric patient with severe Churg-Strauss syndrome refractory to prednisolone."
7.	Azathioprine (Imuran)FDA LinkGeneric 08/19/2005 - "The development of rheumatoid arthritis during remission achieved with azathioprine in Churg-Strauss syndrome of four-year duration is very rare. " 08/01/2010 - "Azathioprine-associated hypersensitivity reaction in a patient with Churg Strauss vasculitis." 12/10/1974 - "[Effective use of azathioprine in a case of polyarteritis nodosa with recurrent intestinal perforation: a distinction between polyarteritis nodosa and allergic granulomatous angiitis]." 02/01/2003 - "The diagnosis of Churg-Strauss syndrome was established, and she was treated with an increased dose of azathioprine and a slowly tapering prednisone regimen. " 11/01/1992 - "The two patients with Churg Strauss syndrome required azathioprine in addition to long-term prednisolone. "
8.	Granulomatous allergic angiitisIBA 06/01/2009 - "Churg-Strauss syndrome (CSS)(allergic granulomatosis)." 07/01/2001 - "Allergic granulomatosis secondary to a limited form of Churg-Strauss syndrome." 03/01/1989 - "Diagnosis was made by means of cutaneous biopsy showing a necrotic granulomatous vasculitic lesions with eosinophilic infiltration and giant cells, consistent with allergic granulomatosis of Churg-Strauss syndrome. " 05/17/1987 - "[Allergic granulomatosis and vasculitis (Churg-Strauss syndrome)]." 06/01/1986 - "Allergic angiitis and granulomatosis (Churg-Strauss syndrome): report of a case with massive thymic involvement in a nonasthmatic patient."
9.	rituximab (Mabthera)FDA Link 01/01/2013 - "Rituximab in the treatment of refractory or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)." 01/01/2013 - "Treatment-resistant Churg-Strauss syndrome: progression after five years using rituximab." 09/01/2011 - "Rituximab for the treatment of Churg-Strauss syndrome with renal involvement." 01/01/2011 - "Churg-Strauss syndrome successfully treated with rituximab." 06/01/2010 - "Successful use of rituximab in a patient with Churg-Strauss syndrome and refractory central nervous system involvement."
10.	Prednisone (Sone)FDA LinkGeneric 08/01/2013 - "A diagnosis of Churg-Strauss syndrome was ultimately made, and the patient was treated with high-dose prednisone therapy. " 11/04/2010 - "Patient was diagnosed of Churg Strauss Syndrome and initial treatment of prednisone was introduced. " 09/01/2003 - "After the diagnosis of Churg-Strauss syndrome, the patient remained symptom free with a normal ejection fraction for 15 months while taking prednisone." 02/01/2003 - "The diagnosis of Churg-Strauss syndrome was established, and she was treated with an increased dose of azathioprine and a slowly tapering prednisone regimen. " 03/01/2013 - "Complete remission of coronary vasculitis in Churg-Strauss Syndrome by prednisone and cyclophosphamide."

Therapies and Procedures

1.	Remission Induction 06/01/2007 - "Treatment for Churg-Strauss syndrome: induction of remission and efficacy of intravenous immunoglobulin therapy." 05/01/2008 - "Interferon-alpha appears to be an effective and well-tolerated treatment for induction of remission in patients with refractory Churg-Strauss syndrome." 05/01/2008 - "This study was carried out to examine the safety and efficacy of interferon-alpha for induction of remission in patients with refractory Churg-Strauss syndrome. " 01/01/2010 - "Interferon-alpha has been successfully used for induction of remission in patients with Churg-Strauss syndrome, but data on its ability to prevent relapses and its safety during long-term use are lacking. " 01/01/2004 - "Churg Strauss syndrome--successful induction of remission with methotrexate and unexpected high cardiac and pulmonary relapse ratio during maintenance treatment."
2.	Plasmapheresis 05/01/1985 - "In the case report the typical symptoms of Churg-Strauss syndrome, the therapeutic possibilities (immunosuppression, plasmapheresis) and the differential diagnosis are discussed." 12/01/2004 - "Complete clinical and functional recovery with a long term stable remission and a low incidence of side effects can be achieved by intravenous immunoglobulin associated with plasmapheresis in patients with Churg-Strauss syndrome." 12/01/2004 - "To study the long term effectiveness of intravenous immunoglobulin and plasmapheresis associated with prednisone and cyclophosphamide in Churg-Strauss syndrome. "
3.	Plasma Exchange 11/01/1995 - "To define the most effective treatment for severe polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS) and to investigate the indication for plasma exchange treatment. " 09/01/1995 - "Churg-Strauss syndrome with peripheral polyneuropathy refractory to steroideal and immunosuppressive therapy successfully treated with plasma exchange." 01/01/2011 - "Plasma exchange in the treatment of Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and renal limited vasculitis." 04/01/2003 - "In polyarteritis nodosa without HBV infection, Wegener's granulomatosis, Churg-Strauss syndrome and in other systemic diseases vasculitides, plasma exchanges can help to control or attenuate the disease. " 02/01/1992 - "Lack of superiority of steroids plus plasma exchange to steroids alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome. "
4.	Aftercare (After-Treatment) 03/01/2010 - "His symptoms and typical echocardiographic findings markedly improved within 2 months after treatment for Churg-Strauss syndrome." 09/01/2005 - "Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients." 08/01/2005 - "Leucoencephalopathy after treatment of Churg-Strauss syndrome with interferon {alpha}." 02/20/2002 - "[Churg-Strauss syndrome after treatment with Singulair (montelukast)]."
5.	Kampo Medicine (Kampo) 01/01/2010 - "Based on the tenets of traditional Chinese medicine (TCM) theory, Kampo medicines were selected and applied to two cases of Churg-Strauss syndrome and Henoch-Shönlein purpura. " 01/01/2010 - "Kampo medicines helped clear the persistent abnormal symptoms and laboratory findings of vasculitis syndromes, Churg-Strauss syndrome and Henoch-Shönlein purpura, which had responded inadequately to the conventional therapies administered." 01/01/2010 - "Kampo medicines improved blood test and QOL in two vasculitis cases of Churg-Strauss syndrome and Henoch-Shönlein purpura after inadequate treatment with conventional therapies."