Churg-Strauss Syndrome Leading to Small Bowel Infarction: An Unusual Case of Abdominal Pain in a Young Patient

A 33-year-old man with a history of severe asthma presented to the emergency department with a week-long history of severe unrelenting abdominal pain, nausea and decreased appetite. He was admitted to hospital, and routine gastrointestinal investigations were performed, which did not elucidate the cause of his abdominal pain. Exploratory laparotomy demonstrated patchy infarction of the entire small bowel, characteristic of Churg-Strauss syndrome. The patient subsequently underwent 12 separate laparotomies to salvage surviving small bowel. The patient is maintained on total parenteral nutrition.

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A Case of Montelukast-Induced Churg-Strauss Syndrome Associated with Liver Dysfunction

Case Reports in Hepatology ◽

10.1155/2011/412524 ◽

2011 ◽

Vol 2011 ◽

pp. 1-4

Author(s):

Keiji Matsui ◽

Kosuke Nishijima

Keyword(s):

Liver Dysfunction ◽

Carnitine Deficiency ◽

Acid Oxidation ◽

Fatty Change ◽

Churg Strauss Syndrome ◽

History Of ◽

Strauss Syndrome ◽

Leukotriene Receptor ◽

The Relationship ◽

Churg Strauss

A 64-year-old woman was admitted to hospital due to protracted diarrhea and liver dysfunction. The patient was diagnosed as Churg-Strauss syndrome (CSS) due to asthma, paranasal sinusitis, hypereosinophilia, and polyneuropathy. There was a history of taking montelukast, a leukotriene receptor antagonist (LTRA), which is thought to have some relationship with CSS. The liver biopsy specimen showed eosinophilic infiltration and centrolobular fatty change. In this paper, we review the relationship between LTRA and CSS. Several lines of evidence suggest that leukotriene plays an important role in maintaining neural tissues. We also review the potential relationship between centrolobular fatty change and pivoxil-containing antibiotics, which was prescribed for sinusitis before admission. Carnitine deficiency induced by pivoxil-containing agents may cause impaired fatty acid oxidation in mitochondria.

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EARLY MUSCLE AFFECTION IN CHURG–STRAUSS SYNDROME

Journal of Musculoskeletal Research ◽

10.1142/s021895770500145x ◽

2005 ◽

Vol 09 (01) ◽

pp. 53-58

Author(s):

Josef Finsterer ◽

Christine Haberler

Keyword(s):

Skeletal Muscle ◽

Muscle Involvement ◽

Churg Strauss Syndrome ◽

Blood Sedimentation ◽

Epitheloid Cells ◽

Blood Sedimentation Rate ◽

Dominant Feature ◽

History Of ◽

Strauss Syndrome ◽

Churg Strauss

Purpose: Skeletal muscle affection already in the eosinophilic stage of Churg–Strauss syndrome (CSS) is unusual. Case report: In a 36-year-old woman with a history of recurrent bronchial infections since childhood, recurrent poly-sinusitis and nasal polyps, and bronchial asthma, easy fatigability, weakness of the lower arms and lower legs, generalized myalgias with predominance of the lower arms, recurrent vertigo and falls developed. There was recurrently elevated creatine-kinase, glutamate-oxalate-transaminase, aldolase, lactate-dehydrogenase, eosinophilic granulocyte-count, thrombocyte-count, cholesterol, and blood sedimentation rate. Histologically polyps showed diffuse infiltrations of eosinophils exclusively. Muscle biopsy revealed grouped atrophic fibers, necrotic fibers, rarely regenerating fibers, epitheloid cells and rarely perimysial eosinophilic granulozytes. According to established criteria, CSS was diagnosed. Conclusions: This case shows that affection of the skeletal muscle in CSS, may manifest as easy fatigability, myalgias, and recurrently elevated CK, and that skeletal muscle involvement may be a dominant feature not only of the vasculitic but also of the eosinophilic stage of CSS.

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Unusual presentation of temporal bone involvement in Churg–Strauss syndrome

The Journal of Laryngology & Otology ◽

10.1017/s0022215107007232 ◽

2007 ◽

Vol 122 (4) ◽

pp. 425-427 ◽

Cited By ~ 7

Author(s):

M Martinez Del Pero ◽

D Moffat ◽

H Sudhoff

Keyword(s):

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Otitis Media ◽

Sudden Onset ◽

Sensorineural Hearing ◽

Churg Strauss Syndrome ◽

First Case ◽

History Of ◽

Strauss Syndrome ◽

Churg Strauss

AbstractObjective:To present a case of a 60-year-old male with a history of sudden onset sensorineural hearing loss due to Churg–Strauss syndrome.Case report:The patient had a 20-year history of asthma and recurrent right otitis media and a nasal polypectomy four years prior to presenting with ear symptoms. Ear, nose and throat involvement is common in Churg–Strauss syndrome, usually manifesting as allergic rhinitis and chronic rhinosinusitis with or without polyps.Conclusions:Otolaryngologists play an important role in making an early diagnosis of this disease. To our knowledge this is the first case of Churg–Strauss syndrome primarily presenting with otological pathology: left sensorineural hearing loss and right otitis media.

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La granulomatosi eosinofilica con poliangioite: dalla pelle al cuore

Medico e Bambino ◽

10.53126/meb39562 ◽

2020 ◽

Vol 39 (9) ◽

pp. 569-574

Author(s):

Matteo Pavan ◽

Anna Agrusti ◽

Andrea Trombetta ◽

Serena Pastore ◽

Alberto Tommasini ◽

...

Keyword(s):

Granulomatosis With Polyangiitis ◽

Immunosuppressive Treatment ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Eosinophilic Inflammation ◽

Peripheral Eosinophilia ◽

Churg Strauss Syndrome ◽

History Of ◽

Strauss Syndrome ◽

Eosinophilic Granulomatosis ◽

Churg Strauss

Key words: Churg-strauss syndrome, Eosinophilic granulomatosis with polyangiitis in childhood, Vasculitis, Asthma, Hypereosinophilia Background - Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, is an extremely rare systemic vasculitis in the paediatric population. The hallmarks of eosinophilic granulomatosis with polyangiitis are a long history of asthma and peripheral eosinophilia with eosinophilic inflammation that may involve several organs. Findings - The paper reports the clinical characteristics, courses, and outcomes of the four patients diagnosed with eosinophilic granulomatosis with polyangiitis at IRCCS Burlo Garofolo (Trieste, Italy) from 1996 to 2015. The mean age at diagnosis was 11.5 years. All the patients presented a history of asthma and peripheral eosinophilia at diagnosis. 3/4 of the children presented upper airway and pulmonary disease. Skin and heart involvement was present in half of the patients, whereas gastrointestinal and neurological symptoms were reported in 25% of the cases. When performed, tissue biopsy revealed eosinophilic inflammation in all the cases. Anti-neutrophil cytoplasmic antibodies were negative in 66% patients. One young child died shortly after presentation, one remitted after immunosuppressive treatment and two patients needed low-dose corticosteroid therapy to maintain the remission. Conclusion - Comparison with an updated review of the series and cases of childhood-onset eosinophilic granulomatosis with polyangiitis reported in the literature showed similar demographic characteristics, clinical features and outcomes. Cardiac disease represents the poorer prognostic factor, leading to the 60% of the deaths reported.

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Severe acute multineuropathy in Churg-Strauss syndrome in a patient with a history of melanoma

Clinical Neuropathology ◽

10.5414/npp28125 ◽

2009 ◽

Vol 28 (03) ◽

pp. 125-128 ◽

Cited By ~ 4

Author(s):

M.E. Fruguglietti ◽

L. Napoli ◽

M. Sciacco ◽

M. Ripolone ◽

M. Serafini ◽

...

Keyword(s):

Churg Strauss Syndrome ◽

History Of ◽

Strauss Syndrome ◽

Churg Strauss

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Successful identical-twin living donor small bowel transplant for necrotizing enterovasculitis secondary to Churg-Strauss syndrome

Transplant International ◽

10.1111/j.1432-2277.2006.00316.x ◽

2006 ◽

Vol 19 (7) ◽

pp. 594-597 ◽

Cited By ~ 13

Author(s):

Stefano Schena ◽

Giuliano Testa ◽

Suman Setty ◽

Herand Abcarian ◽

Enrico Benedetti

Keyword(s):

Small Bowel ◽

Living Donor ◽

Identical Twin ◽

Churg Strauss Syndrome ◽

Small Bowel Transplant ◽

Strauss Syndrome ◽

Churg Strauss

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Churg‐Strauss syndrome revealed by acute abdominal pain

Pediatric Pulmonology ◽

10.1002/ppul.1093 ◽

2001 ◽

Vol 32 (1) ◽

pp. 92-94 ◽

Cited By ~ 6

Author(s):

M. Berlioz ◽

V. Triolo ◽

N. Sirvent ◽

M. Albertini

Keyword(s):

Abdominal Pain ◽

Acute Abdominal Pain ◽

Churg Strauss Syndrome ◽

Strauss Syndrome ◽

Churg Strauss

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Clinicopathological Features of Churg-Strauss Syndrome With Severe Nerve Degeneration: A Case Report

Medicina ◽

10.3390/medicina48050035 ◽

2012 ◽

Vol 48 (5) ◽

pp. 35

Author(s):

Jing Xiang ◽

Yu Zhao ◽

Jian Fu ◽

Xiao Cheng ◽

Qin Xue ◽

...

Keyword(s):

Acute Asthma ◽

Lower Limbs ◽

Nerve Degeneration ◽

Unknown Etiology ◽

Churg Strauss Syndrome ◽

Multiple Systems ◽

Tissue Eosinophilia ◽

History Of ◽

Strauss Syndrome ◽

Churg Strauss

Churg-Strauss syndrome (CSS) is a rare autoimmune vasculitis of unknown etiology that involves small- and medium-sized blood vessels. Its onset is thought to be associated with adult-onset asthma, and vasculitis typically involves vessels in the lungs. However, due to increased blood and tissue eosinophilia, vasculitis may result in the involvement multiple systems of (neurological, skin, etc.). We report a case of CSS with manifestations that included skin purpura and severe peripheral nerve degeneration in a 56-year-old woman with a recent history of asthma. After the treatment with methylprednisolone and standard immunosuppressive therapy, her rashes resolved, there were no acute asthma attacks, and the numbness in her lower limbs improved.

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