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

2009 ◽  
Vol 28 (03) ◽  
pp. 125-128 ◽  
Author(s):  
M.E. Fruguglietti ◽  
L. Napoli ◽  
M. Sciacco ◽  
M. Ripolone ◽  
M. Serafini ◽  
...  
2011 ◽  
Vol 2011 ◽  
pp. 1-4
Author(s):  
Keiji Matsui ◽  
Kosuke Nishijima

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.


2005 ◽  
Vol 09 (01) ◽  
pp. 53-58
Author(s):  
Josef Finsterer ◽  
Christine Haberler

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.


2007 ◽  
Vol 122 (4) ◽  
pp. 425-427 ◽  
Author(s):  
M Martinez Del Pero ◽  
D Moffat ◽  
H Sudhoff

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.


2020 ◽  
Vol 39 (9) ◽  
pp. 569-574
Author(s):  
Matteo Pavan ◽  
Anna Agrusti ◽  
Andrea Trombetta ◽  
Serena Pastore ◽  
Alberto Tommasini ◽  
...  

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.


Medicina ◽  
2012 ◽  
Vol 48 (5) ◽  
pp. 35
Author(s):  
Jing Xiang ◽  
Yu Zhao ◽  
Jian Fu ◽  
Xiao Cheng ◽  
Qin Xue ◽  
...  

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.


1995 ◽  
Vol 108 (2) ◽  
pp. 200-202 ◽  
Author(s):  
Mayumi Ogasawara ◽  
Shoji Kajiura ◽  
Hiroshi lnagaki ◽  
Haruki Sasa ◽  
Koji Aoki ◽  
...  

1998 ◽  
Vol 12 (3) ◽  
pp. 219-222 ◽  
Author(s):  
Sunil Sookram ◽  
Camille Hancock-Friesen ◽  
JP Ferguson ◽  
Terry Sosnowski ◽  
Laurie JM Russell

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.


1970 ◽  
Vol 10 (2) ◽  
Author(s):  
Lauren K. King, MBBS, MSc. ◽  
Julie Wright, MD ◽  
Christian Pagnoux, MD, MPH ◽  
Janice L. Kwan MD, MPH

Eosinophilia with granulomatosis and polyangiitis (EGPA, previously called Churg-Strauss syndrome or allergic granulomatosis and angiitis) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis affecting small-sized vessels, which typically occurs in patients with a history of allergic rhinitis or asthma. The most serious cases arise from the involvement of other organ systems, notably the cardiovascular system. Importantly, patients may first exhibit non-specific manifestations such as malaise, fever, anorexia, and weight loss. This variable presentation and the potential for multi-organ involvement can lengthen time to diagnosis and delay treatment.We describe a patient who presented with progressive myalgias on the background of recently diagnosed rhinosinusitis who was ultimately diagnosed with EGPA. This case is meant to alert general internists to the diagnostic challenges of EGPA.


Author(s):  
Sharim Fathima ◽  
Jisy S. R.

<p class="abstract">Churg Strauss syndrome (CSS) also known as eosinophilic granulomatosis with polyangitis (EGPA), is a rare disease manifested by hypereosinophilia, vasculitis and extravascular granuloma. We report a case of 72 year old male with history of asthma and allergic rhinitis who presented with non-healing ulcer on dorsum of left middle finger and petechial lesions on forearms. Investigations revealed marked eosinophilia, elevated IgE, negative antineutrophil cytoplasmic antibodies (ANCA), histopathologic examination showed granulomatous vasculitis and CSS was diagnosed using ACR criteria. We report this case because of rarity of disease and the importance of recognizing similar presentation for early diagnosis and treatment of this life threatening syndrome.</p>


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