scholarly journals A Presentation of Massive Hemoptysis in a Patient with Churg-Strauss Syndrome

2014 ◽  
Vol 21 (6) ◽  
pp. 337-340
Author(s):  
Fadi Hikmat ◽  
David B Pearse ◽  
Mahendra Damarla
Keyword(s):  
Computed Tomography ◽  
Present Report ◽  
Sudden Onset ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Massive Hemoptysis ◽  
Bronchial Arteries ◽  
Churg Strauss Syndrome ◽  
Strauss Syndrome ◽  
Churg Strauss

Given that Churg-Strauss syndrome is a systemic small-vessel vasculitis, it is not usually considered in patients who present with massive hemoptysis, which is typically caused by bronchiectasis, cancer or, in some cases, aberrant bronchial arteries. This article, however, describes a novel case involving a 50-year-old Churg-Strauss patient who presented with sudden-onset massive hemoptysis. Details of the physical examination, laboratory investigations and several imaging studies, including computed tomography, bronchoscopy and three-dimensional imaging, are presented.Churg-Strauss syndrome (CSS) is a systemic small-vessel vasculitis. When involving the lungs, small-vessel vasculitides typically cause capillaritis, leading to diffuse alveolar hemorrhage and submassive hemoptysis. In contrast, massive hemoptysis primarily originates from the bronchial arteries; therefore, small-vessel vasculitis is not considered when a patient presents with massive hemoptysis. The authors describe a patient with CSS who presented with the novel finding of massive hemoptysis. Computed tomography scans lacked alveolar infiltrates and bronchoalveolar lavage lacked hemosiderin-laden macrophages. Bronchoscopy demonstrated a raised mucosal lesion in the right mainstem bronchus and computed tomography angiogram revealed aberrant dilated bronchial arteries underlying the same region, suggesting this as the source of the hemoptysis. To the authors’ knowledge, the present report describes the first reported case of CSS to present with massive hemoptysis with likely involvement of the bronchial arterial circulation. CSS should be considered in patients with unexplained massive hemoptysis.

scholarly journals ANCA-negative Churg-Strauss Syndrome

2016 ◽  
Vol 6 (2) ◽  
pp. 178
Author(s):  
Syed Jamil Abdal ◽  
Md. Zilan Miah Sarker ◽  
Syed Md. Monowar Ali ◽  
A. K. M. Motiur Rahman Bhuiyan ◽  
Md. Nahiduzzaman Shazzad
Keyword(s):  
Immunosuppressive Agents ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Unknown Etiology ◽  
Primary Therapy ◽  
Churg Strauss Syndrome ◽  
Systemic Glucocorticoids ◽  
Strauss Syndrome ◽  
Bangladeshi Population ◽  
Churg Strauss

A rare and a disease of unknown etiology, Churg-Strauss syndrome (CSS) is a granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia, which is initially described by Churg and Strauss in 1951. Because of its clinical and pathological features that overlap with those of the other anti-neutrophil antibody (ANCA)-associated systemic vasculitides (AASVs) and now the disease is classified as AASVs. The ANCA status may dictate the clinical phenotype. ANCA-positive patients are significantly more likely to have disease manifesta­tions associated with small-vessel vasculitis, including oecrotising glomemlonephritis, mononeuritis and purpura, whereas ANCA-negative cases predominantly likely to have cardiac and lung involvement. The objective of this case report is to point out the possibility of vasculitic rash in ANCA-negative CSS in a 35-year-old man and the disease rarely occurs in Bangladeshi population. We analyze the history, clinical examinations and relevant investigations related to the patient to establish the diagnosis in our department. The clinical scenario and biopsy help us to attain the diagnosis. But due to unavailability of patients' cohort we have limitations of comparison of ANCA status in Bangladeshi populations. Though ANCA-positive and ANCA-negative CSS differ phenotypically, primary therapy for both the conditions is systemic glucocorticoids. Additional immunosuppressive agents like cyclophosphamide, mycophenolate mofetil, azathioprine, rituxin1ab are occasionally added in patients with more advanced or refractory disease.

Pulmonary findings in Churg–Strauss syndrome in chest X-rays and high resolution computed tomography at the time of initial diagnosis

2010 ◽  
Vol 29 (10) ◽  
pp. 1127-1134 ◽  
Author(s):  
Wojciech Szczeklik ◽  
Barbara Sokołowska ◽  
Lucyna Mastalerz ◽  
Piotr Grzanka ◽  
Jacek Górka ◽  
...  
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Initial Diagnosis ◽  
X Rays ◽  
High Resolution Computed Tomography ◽  
Churg Strauss Syndrome ◽  
Strauss Syndrome ◽  
Churg Strauss

Occam’s razor reveals a hidden Churg-Strauss syndrome

Open Medicine ◽  
2012 ◽  
Vol 7 (3) ◽  
pp. 358-361
Author(s):  
Raffaella Nenna ◽  
Maurizio Mennini ◽  
Laura Petrarca ◽  
Gerarda Mastrogiorgio ◽  
Fabio Magliocca ◽  
...  
Keyword(s):  
Mhc Class Ii ◽  
Nyha Class ◽  
Class Ii ◽  
Sudden Onset ◽  
Left Ventricular ◽  
Churg Strauss Syndrome ◽  
American College Of Rheumatology ◽  
Duodenal Lesions ◽  
Strauss Syndrome ◽  
Churg Strauss

AbstractA 28 year-old caucasian lady, with nine years of uncontrolled bronchial asthma, rhinosinusitis and mild upper limb paresthesia, came to our attention to be followed for coeliac disease (CD). She had a biopsy performed elsewhere which proved the diagnosis five years before. Since there was no clinical improvement on a strict gluten-free diet, we re-evaluated the slides of her duodenal biopsies and we found an overestimation of the duodenal lesions due to the wrong orientation of the specimens. Moreover, she had never had positive CD-related antibodies and she was negative for DQ2/DQ8 MHC Class II heterodimers. Months later, she referred she was suffering from diffuse joint pain, epistaxis and a substantial weight loss. A few days later she was hospitalized because of a sudden onset of dyspnea, peripheral edema and pleural effusion. Her echocardiogram showed global left ventricular hypokenesia with an ejection fraction of 24%. The patient was discharged with a diagnosis of dilated cardiomyopathy and NYHA Class II. After a large spectrum of haematological exams, the diagnosis of Churg Strauss Syndrome (CSS), a rare multisystemic small-vessel necrotizing vasculitis, was confirmed by the presence of four/five out of six diagnostic American College of Rheumatology classification criteria (Asthma, Eosinophilia >10%, Neuropathy, Non-fixed pulmonary infiltrates, Paranasal sinus abnormality and Biopsy containing a blood vessel with extravascular eosinophils). Our patient had been under-diagnosed by pulmonologist and by gastroenterologists although she presented the criteria required for CSS diagnosis. Our case report emphasizes that often seemingly unrelated symptoms can be caused by a single rare clinical complex.

Unusual presentation of temporal bone involvement in Churg–Strauss syndrome

2007 ◽  
Vol 122 (4) ◽  
pp. 425-427 ◽  
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.

scholarly journals ANCA - Positive Churg Strauss Syndrome with Necrotizing Crescentic glomerulonephritis

JMS SKIMS ◽  
2009 ◽  
Vol 12 (1) ◽  
pp. 21-23
Author(s):  
A R Reshi ◽  
I A Wani ◽  
K A Banday ◽  
Iqbal Lone ◽  
M M Wani ◽  
...  
Keyword(s):  
Renal Failure ◽  
Renal Biopsy ◽  
Crescentic Glomerulonephritis ◽  
Renal Involvement ◽  
Eosinophilic Infiltration ◽  
Small Vessel Vasculitis ◽  
Churg Strauss Syndrome ◽  
Necrotizing Crescentic Glomerulonephritis ◽  
Strauss Syndrome ◽  
Churg Strauss

Churg Strauss Syndrome (CSS) is an ANCA associated small vessel vasculitis with eosinophilic infiltration. Renal involvement is frequent, although usually mild. We report a case of CSS with renal failure. Renal biopsy revealed necrotizing cresentic glomerulonephritis, eosonophilic arteritis and diffuses eosinophilic infiltration. Patient was successfully treated with combination of corticosteroids and cyclophosphamide. (JMed Sci 2009;12(1):21-23)

Primary vasculitides

2011 ◽  
pp. 405-430 ◽  
Author(s):  
Alan J. Hakim ◽  
Gavin P.R. Clunie ◽  
Inam Haq
Keyword(s):  
Rheumatoid Arthritis ◽  
Kawasaki Disease ◽  
Polyarteritis Nodosa ◽  
Polymyalgia Rheumatica ◽  
Large Vessel ◽  
Small Vessel Vasculitis ◽  
Churg Strauss Syndrome ◽  
Established Disease ◽  
Strauss Syndrome ◽  
Churg Strauss

Introduction 406 Large-vessel vasculitis 409 Takayasu's arteritis 410 Polymyalgia rheumatica and giant cell arteritis 412 Polyarteritis nodosa 416 Wegener's granulomatosis 418 Other forms of antineutrophil cystoplasmic antibody-associated vasculitides: microscopic polyangiitis and the Churg–Strauss syndrome 422 Small-vessel vasculitis 426 Kawasaki disease 430 • The vasculitides are a heterogeneous group of relatively uncommon diseases that can arise as primary conditions or secondary to an established disease such as rheumatoid arthritis (RA) (...

scholarly journals Nonasthmatic Churg–Strauss syndrome superimposed on chronic pyelonephritis: a case report

2021 ◽  
Vol 49 (9) ◽  
pp. 030006052110483
Author(s):  
Aleksandra Kezić ◽  
Stojanka Ristić ◽  
Maja Životić ◽  
Jasmina Marković-Lipkovski ◽  
Svetlana Kovačević ◽  
...  
Keyword(s):  
Drug Allergy ◽  
Interstitial Fibrosis ◽  
Chronic Pyelonephritis ◽  
Eosinophil Infiltration ◽  
Small Vessel Vasculitis ◽  
Creatinine Concentration ◽  
Churg Strauss Syndrome ◽  
Atypical Form ◽  
Strauss Syndrome ◽  
Churg Strauss

Churg–Strauss syndrome (CSS) is a granulomatous small-vessel vasculitis. Asthma is seen in the majority of patients with CSS, but atypical nonasthmatic forms of CSS are also being recognized. We herein describe a 67-year-old woman with a history of chronic pyelonephritis and drug allergy reactions who was admitted to our hospital because of worsening renal function preceded by fever, purpura, sinusitis, and a positive urine culture that confirmed a urinary infection. She was initially treated with pipemidic acid for 7 days, followed by clarithromycin for sinusitis. Laboratory tests on admission showed an absolute eosinophil count of 1750 cells/µL and serum creatinine concentration of 4.72 mg/dL. Urine and blood cultures showed no growth. Kidney biopsy revealed crescent formations with diffuse interstitial fibrosis and foci of eosinophil infiltration. An atypical form of CSS was diagnosed based on tissue eosinophilia, peripheral eosinophilia, and sinusitis. Intravenous methylprednisolone and cyclophosphamide pulse therapy together with hemodialysis treatment improved the patient’s clinical condition but did not resolve the kidney damage. The onset of an atypical form of CSS in our patient manifested as symptoms and signs mimicking those of chronic pyelonephritis and drug allergy reactions. The patient’s chronic kidney disease finally progressed to dialysis dependence.

Peripheral Neuropathy in a Patient with Churg-Strauss Syndrome - A case report -

2007 ◽  
Vol 20 (2) ◽  
pp. 208
Author(s):  
Jun Hwa Lee ◽  
Jeong Hyun Lee ◽  
Young Kwon Go ◽  
Won Hyung Lee
Keyword(s):  
Case Report ◽  
Peripheral Neuropathy ◽  
Churg Strauss Syndrome ◽  
Strauss Syndrome ◽  
Churg Strauss
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