Churg-Strauss syndrome with necrosis of toe tips.

Published on Jun 1, 2011in Acta Medica Okayama0.642
· DOI :10.18926/AMO/46635
Koichi Waseda9
Estimated H-index: 9
Yasushi Tanimoto21
Estimated H-index: 21
(Okayama University)
+ 7 AuthorsMitsune Tanimoto68
Estimated H-index: 68
Churg-Strauss syndrome (CSS) is a granulomatous necrotizing vasculitis of unknown etiology associated with bronchial asthma. Despite affecting small to medium-sized vessels, necrosis of the digits due to vasculitis is extremely rare. We report a case of CSS with necrosis of the toe tips. A 37-year-old woman with asthma, who had been diagnosed with CSS 2 years ago, was admitted to our hospital with an exacerbation of CSS. The patient had a high grade fever and complained of abdominal pain and numbness of the lower extremities. Blood examination revealed marked eosinophilia. The fever pattern, abdominal pain and blood eosinophilia showed improvement by combination treatment with prednisolone and cyclophosphamide. However, the color of her right toe tips changed, and necrosis finally resulted despite antithrombotic therapy. Arteriography showed narrowing of the dorsalis pedis artery and of the more peripheral arteries of her right leg. Stump plasty with negative pressure dressing therapy for the toe tips, but not amputation, was done to preserve the leg function. While numbness of the extremities remained, no recurrence of necrosis was seen. Clinicians need to be aware that rare complications of CSS, including necrosis of the digits, can occur.
📖 Papers frequently viewed together
1 Citations
4 Citations
#1Sophia Kim (Boston Children's Hospital)H-Index: 1
#1Sophia Kim (Boston Children's Hospital)H-Index: 2
Last. Michael E. Wechsler (Brigham and Women's Hospital)H-Index: 55
view all 5 authors...
Background Treatments for Churg-Strauss syndrome (CSS), a rare eosinophilic vasculitis characterized by asthma, sinusitis, peripheral eosinophilia, pulmonary infiltrates, and tissue infiltration, are limited by toxicity or poor efficacy. Levels of IL-5, a cytokine regulating eosinophils, can be increased in patients with CSS. Mepolizumab, a humanized monoclonal anti–IL-5 antibody, decreases steroid requirements in patients with non-CSS hypereosinophilic syndromes. Objective The purpose of this s...
212 CitationsSource
#1Susan Bibby (Wellington Management Company)H-Index: 6
#2Bridget HealyH-Index: 9
Last. Richard BeasleyH-Index: 103
view all 6 authors...
Background The possible role of leukotriene receptor antagonist (LTRA) therapy in the pathogenesis of Churg-Strauss syndrome (CSS) is uncertain. The aim was to examine the association between LTRA therapy and CSS in cases registered in the FDA Adverse Event Reporting System (AERS) database. Methods All cases of suspected drug-induced CSS reported to the AERS database between November 1997 and April 2003 were reviewed. Subjects in whom LTRAs were the suspected medication and sufficient documentat...
74 CitationsSource
#1Jean-Emmanuel Kahn (university of lille)H-Index: 28
Last. Olivier BletryH-Index: 48
view all 7 authors...
93 CitationsSource
Epidemiologic evidence has shown that the worldwide prevalence of asthma is increasing. The leukotriene receptor antagonists (LTRAs) represent a new class of therapy for asthma. They have been developed in the last decade and play a pivotal steroid-sparing role in treating the inflammatory component of asthma. Consequently, reports of Churg-Strauss syndrome (CSS), a rare form of systemic vasculitis, have been recognized as a potential side effect in individuals with moderate to severe asthma on ...
14 CitationsSource
#1Yuichiro OtaniH-Index: 2
#2Saburo AnzaiH-Index: 7
Last. Shigeo YokoyamaH-Index: 29
view all 9 authors...
We report a case of Churg-Strauss syndrome (CSS) with necrosis of the fingers and toes and liver infarction. A 59-year-old man with asthma suddenly noticed that his fingers and toes felt unusually cold. This condition worsened progressively, and some digits became necrotic within several weeks. Laboratory studies revealed hypereosinophilia and an extremely elevated serum level of IgE. Digital subtraction angiography of the extremities revealed extensive irregular narrowing of small and medium-si...
23 CitationsSource
#1Imre Noth (U of C: University of Chicago)H-Index: 42
#2Mary E. Strek (U of C: University of Chicago)H-Index: 31
Last. Alan R. Leff (U of C: University of Chicago)H-Index: 56
view all 3 authors...
Summary Churg-Strauss syndrome is a rare diffuse vasculitis that is almost invariably accompanied by severe asthma. Although overall prognosis is good, and treatment with prednisone alone or in combination with immunosuppressive drugs is usually successful, severe asthma typically persists. Diffuse organ involvement of Churg-Strauss syndrome, especially cardiovascular and rare involvement of the CNS and renal system, suggests a poorer prognosis than usual, and can be fatal. The cause of Churg-St...
450 CitationsSource
#1Nathalie MémainH-Index: 1
#2Bandt Michel DeH-Index: 1
Last. Olivier MeyerH-Index: 61
view all 5 authors...
Churg-Strauss syndrome (CSS) is characterized by small vessel vasculitis and extravascular granulomas. The American College of Rheumatology classification criteria for CSS include asthma, eosinophili, and clinical manifestation of vasculitis. Gastrointestinal (GI) manifestations occur in 30% of patients, but are inaugural in only 16%. They denote vasculitis of the stomach and small bowel wall, and consist in protean, nonspecific pain. GI involvement is of adverse prognostic significance in CSS. ...
15 Citations
#1Yumiko IkemotoH-Index: 11
#2Urara KohderaH-Index: 10
Last. Yohnosuke KobayashiH-Index: 30
view all 6 authors...
21 CitationsSource
#1Shigeki Inui (Osaka University)H-Index: 28
#2Satoshi Itami (Osaka University)H-Index: 60
Last. Kunihiko Yoshikawa (Osaka University)H-Index: 13
view all 4 authors...
A 69-year-old Japanese man suffered from bronchial asthma, atrial fibrillation, general fatigue, high fever, and weight loss of about 5 kg within a month. He also had intermittent claudication, a tingling feeling in his fingers and toes, and an ulcer on his toe. His laboratory data revealed leukocytosis with absolute eosinophilia. The patient was treated with predonisolone 30 mg daily. Although the ulcers healed once, the lesions recurred with tapering predonisolone. The patient visited us becau...
7 CitationsSource
9 Citations
Cited By4
#1Xiaoliang Liu (Sichuan University)H-Index: 6
#2Lin WangH-Index: 1
Last. Chuan WangH-Index: 5
view all 6 authors...
Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis in children. A delayed or missed diagnosis of pediatric EGPA is common, owing to the atypical clinical manifestation and limited recognition of this disorder. The vasculitis in EGPA typically involves small to medium size vessels. Extensive occlusion of arteries in the extremities was being extremely rare and has never been reported in children.
#1Ki Jin Jung (Soonchunhyang University Hospital)H-Index: 5
#1Ki Jin Jung (Soonchunhyang University Hospital)H-Index: 3
Last. Byung-Sung Kim (Soonchunhyang University Hospital)H-Index: 2
view all 7 authors...
We present 4 cases of symmetrical peripheral gangrene (SPG) associated with use of inotropic agent to elevate blood pressure. SPG is a relatively rare phenomenon characterized by symmetrical distal ischemic damage that leads to gangrene of 2 or more sites in the absence of large blood vessel obstruction, where vasoconstriction rather than thrombosis is implicated as the underlying pathophysiology. We present 4 SPG cases of the multiple limbs amputation, associated with inevitable use of inotropi...
5 CitationsSource
#1Naoto AzumaH-Index: 8
#2Satoaki MatobaH-Index: 41
Last. Hajime SanoH-Index: 44
view all 12 authors...
A 62-year-old-man presented to our hospital with complaints of coldness, numbness, pain, weakness and cyanosis on the fingers and toes in March 2010. Laboratory findings revealed marked eosinophilia (46.6%; WBC 20600/μl), an elevation of serum creatine kinase, proteinuria and hematuria. He was diagnosed as hypereosinophilic syndrome (HES) without evidence to support a diagnosis of underlying diseases causing eosinophilia. After the initiation of corticosteroid therapy, peripheral eosinophil coun...
Churg–Strauss syndrome (CSS) is a well established disorder of the blood vessels. However, it is still sometimes not diagnosed early enough to avoid serious complications. It is the later stage that carries the more serious complications. Therefore, we emphasize the ways in which the diagnosis can be made early enough to avoid this stage of the disease. We report a case of CSS that was not diagnosed early and so the patient went on to develop severe cardiac and neurological manifestations. In th...