Limited form of Churg-Strauss syndrome presenting as a mass in the neck

2002 ◽  
Vol 116 (11) ◽  
pp. 966-968 ◽  
Author(s):  
J. W. Moor ◽  
J. U-King Im ◽  
A. W. MacDonald ◽  
E. Whitehead
Keyword(s):  
Diagnostic Criteria ◽  
Systemic Vasculitis ◽  
Large Mass ◽  
Histological Diagnosis ◽  
Churg Strauss Syndrome ◽  
Granulomatous Vasculitis ◽  
Supraclavicular Fossa ◽  
Strauss Syndrome ◽  
Churg Strauss

A 53-year-old man was referred to the ENT department with a large mass in the left supraclavicular fossa. The histological diagnosis showed the mass to have arisen due to a granulomatous vasculitis consistent with Churg-Strauss syndrome (CSS). CSS usually comprises asthma, eosinophilia and systemic vasculitis although limited forms of the disease exist where one of these diagnostic criteria is missing. This is one such case as the patient was non-asthmatic.

Allergic granulomatous vasculitis (Churg-Strauss syndrome)

1981 ◽  
Vol 26 (3) ◽  
pp. 264-270 ◽  
Author(s):  
Robert Modigliani ◽  
Jean-Marie Muschart ◽  
Annie Galian ◽  
Jean-Pierre Clauvel ◽  
Jean-Luc Piel-Desruisseaux
Keyword(s):  
Churg Strauss Syndrome ◽  
Granulomatous Vasculitis ◽  
Strauss Syndrome ◽  
Churg Strauss

scholarly journals Pleural Effusion in Churg-Strauss Syndrome Detected and Successfully Treated at a Tertiary Care Institute: Case Report

2021 ◽  
Vol 6 (3) ◽  
pp. 63-65
Author(s):  
Uday Mahajan ◽  
Deepika Kapil
Keyword(s):  
Pleural Effusion ◽  
Systemic Vasculitis ◽  
Tertiary Care ◽  
Oral Prednisolone ◽  
Churg Strauss Syndrome ◽  
X Ray ◽  
Allergic Rhinitis Patient ◽  
Chief Complaints ◽  
Strauss Syndrome ◽  
Churg Strauss

Churg-Strauss syndrome is a disorder of hypereosinophilia and systemic vasculitis in subjects with asthma and allergic rhinitis. Patient was admitted with chief complaints of cough without expectoration for a duration of three weeks without accompanying fever. X ray revealed pleural effusion ‘right side’. Exudative pleural fluid with eosinophil predominance and peripheral eosinophilia. The patient was further investigated. No history suggestive of any drug use. stool routine microscopy show no parasites. ANA investigated found to be positive in 1: 80 titre in homogenous pattern. Patient was then found to be P-ANCA positive by immunofluorescence microscopy. Patient was successfully treated with oral prednisolone 40 mg for three months and subsequently improved with disappearance of symptoms. Keywords: Churg-Strauss syndrome, Pleural effusion, hypereosinophilia.

scholarly journals Wheezing Beyond Bronchial Asthma

2013 ◽  
Vol 34 (2) ◽  
pp. 48-52
Author(s):  
P Pant ◽  
S Sharma ◽  
R Shrestha ◽  
N Bom ◽  
S Das ◽  
...  
Keyword(s):  
Joint Pain ◽  
Late Onset ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Institute Of Medicine ◽  
Upper Airways ◽  
Churg Strauss Syndrome ◽  
Progressive Dyspnea ◽  
Strauss Syndrome ◽  
Churg Strauss

Churg-Strauss syndrome (CSS) is an eosinophil-associated, small vessel granulomatous vaculitis, characterized by late onset asthma, upper airways disease, eosinophilia, and clinical manifestations of systemic vasculitis. [1,11-13] Diagnosis is mainly clinical with findings of asthma, eosinophilia, rhino sinusitis and signs of vasculitis in major organs. So far to the best of our knowledge reporting of CSS has not been done in Nepal. We here present a case of Churg- Strauss syndrome, a Anti-Neutrophil Cytoplasmic Antibody(ANCA) Associated Vasculities more specifically perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) directed against myeloperoxidase (MPO) in a 25years female patient who was initially evaluated for progressive dyspnea, wheeze, cough, joint pain and fever. Regarding the inference drawn from our case report we suggest that patient presenting with the clinical features of asthma needs further evaluation to unmask previously unrecognized underlying Churg-stauss syndrome. DOI: http://dx.doi.org/10.3126/joim.v34i2.9055 Journal of Institute of Medicine August, 2012; 34:2 48-52

Interleukin-25: a cytokine linking eosinophils and adaptive immunity in Churg-Strauss syndrome

Blood ◽  
2010 ◽  
Vol 116 (22) ◽  
pp. 4523-4531 ◽  
Author(s):  
Benjamin Terrier ◽  
Ivan Bièche ◽  
Thierry Maisonobe ◽  
Ingrid Laurendeau ◽  
Michèlle Rosenzwajg ◽  
...  
Keyword(s):  
T Cells ◽  
Disease Activity ◽  
Adaptive Immunity ◽  
Mononuclear Cells ◽  
Systemic Vasculitis ◽  
Critical Role ◽  
Activated T Cells ◽  
Churg Strauss Syndrome ◽  
Strauss Syndrome ◽  
Churg Strauss

Abstract Churg-Strauss syndrome (CSS) is characterized by systemic vasculitis and blood and tissue eosinophilia. Blood eosinophilia correlates with disease activity, and activated T cells from CSS patients are predominantly T helper 2 (Th2). Interleukin (IL)-25 has been shown to link innate and adaptive immunity by enhancing Th2 cytokine production. We sought to determine the involvement of IL-25 and its receptor IL-17RB in the pathogenesis of CSS. We found increased levels of IL-25 in the serum of active CSS patients (952 ± 697 vs 75 ± 49 pg/mL in inactive patients and 47 ± 6 pg/mL in healthy donors). IL-25 was correlated with disease activity and eosinophil level. Eosinophils were the main source of IL-25, whereas activated CD4+ memory T cells were the IL-17RB–expressing cells in CSS. IL-25 enhanced the production of IL-4, IL-5, and IL-13 by activated peripheral blood mononuclear cells. IL-25 and IL-17RB were observed within the vasculitic lesions of patients with CSS, and IL-17RB colocalized with T cells. Increased expression of IL-17RB, tumor necrosis factor receptor–associated factor 6, and JunB in vasculitic lesions of CSS underscored the IL-25–mediated activation, whereas up-regulation of GATA3 and IL-10 supported Th2 differentiation. Our findings suggest that eosinophils, through the production of IL-25, exert a critical role in promoting Th2 responses in target tissues of CSS.

scholarly journals DIFFICULTIES IN THE EARLY DIAGNOSIS OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURGSTRAUSS SYNDROME) IN THE CLINIC OF INTERNAL DISEASES

2018 ◽  
Vol 4 (2) ◽  
pp. 38-47
Author(s):  
D. V. Cherkashin ◽  
S. A. Turdialieva ◽  
E. A. Mozharovskaya ◽  
O. M. Kudrina ◽  
A. I. Taranov
Keyword(s):  
Early Diagnosis ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Churg Strauss Syndrome ◽  
Therapy Strategy ◽  
Wide Range ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

Systemic vasculitis are characterized by heterogeneity of clinical-immunological forms and determined the need for differential diagnostic search to except a wide range of diseases, such as allergic, infectious, hematological, oncological, which often presents significant difficulties for physicians of various specialties. The article presents clinical observations demonstrating the difficulties of diagnostic search in establishing the diagnosis of systemic vasculitis associated with antineutrophil cytoplasmic antibodies, which include a rare disease — eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome). Carefully collected anamnesis, participation of specialists of different profiles, retrospective analysis of laboratory and instrumental data allowed to verify the diagnosis, to prescribe adequate therapy. The aim of the publication is to discuss the need for early diagnosis of eosinophilic granulomatosis with polyangiitis, which can improve the effectiveness of therapy and improve the overall prognosis for this disease, taking into account modern approaches based on the main provisions of international recommendations that were prepared in 2015 with the participation of leading experts from Europe, USA and Canada and were called to become the basis for choosing a personalized patient therapy strategy.

scholarly journals A case of Churg-Strauss syndrome whose diagnosis was given without the findings of systemic vasculitis and granulomatous angiitis.

Ensho ◽  
2000 ◽  
Vol 20 (2) ◽  
pp. 167-173
Author(s):  
Hiroaki Inamura ◽  
Motohiro Kurosawa ◽  
Rieko Nakagami ◽  
Jun-ichiro Morioka ◽  
Yutaka Mizushima ◽  
...  
Keyword(s):  
Systemic Vasculitis ◽  
Granulomatous Angiitis ◽  
Churg Strauss Syndrome ◽  
Strauss Syndrome ◽  
Churg Strauss

scholarly journals An Abdominal Presentation of Churg-Strauss Syndrome

2010 ◽  
Vol 2010 ◽  
pp. 1-4
Author(s):  
J. R. E. Rees ◽  
P. Burgess
Keyword(s):  
New York ◽  
Systemic Vasculitis ◽  
Granulomatous Inflammation ◽  
Signs And Symptoms ◽  
Lower Limbs ◽  
Churg Strauss Syndrome ◽  
Allergic Granulomatous Angiitis ◽  
Strauss Syndrome ◽  
Discharge From Hospital ◽  
Churg Strauss

Churg-Strauss syndrome is a small and medium vessel vasculitis that is also known as allergic granulomatous angiitis. It most commonly presents with an asthma like symptoms. It was first described in Mount Siani Hospital, New York in 1951 by Jacob Churg and Lotte Stauss and was recognised after the study of a series of 13 patients who had asthma, eosinophilia, granulomatous inflammation necrotising systemic vasculitis and necrotising glomerulonephritis. We describe a case of Churg-Strauss syndrome presenting with abdominal pain and later during the hospital admission a mono-neuritis multiplex syndrome affecting the lower limbs. The patient presented in such an atypical fashion with abdominal signs and symptoms that they required laparotomy and the diagnosis was made after histological examination of tissue taken at the time of surgery. Treatment with immunosuppression and aggressive rehabilitation achieved a progressive recovery which continued on discharge from hospital.

scholarly journals Anti-neutrophil cytoplasmic antibody - negative Churg-Strauss syndrome presenting as non-healing ulcer on finger and associated lichen amyloidosis: a case report

2018 ◽  
Vol 4 (3) ◽  
pp. 437
Author(s):  
Sharim Fathima ◽  
Jisy S. R.
Keyword(s):  
Middle Finger ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Healing Ulcer ◽  
Churg Strauss Syndrome ◽  
Granulomatous Vasculitis ◽  
Life Threatening ◽  
History Of ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

<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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