ANCA-negative Churg-Strauss Syndrome

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.

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

Canadian Respiratory Journal ◽

10.1155/2014/620542 ◽

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.

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Pulmonary manifestations of the Churg–Strauss syndrome and related idiopathic small vessel vasculitis syndromes

Current Opinion in Pulmonary Medicine ◽

10.1097/mcp.0b013e3281eb8edb ◽

2007 ◽

Vol 13 (5) ◽

pp. 445-450 ◽

Cited By ~ 11

Author(s):

W Michael Alberts

Keyword(s):

Small Vessel ◽

Small Vessel Vasculitis ◽

Churg Strauss Syndrome ◽

Pulmonary Manifestations ◽

Strauss Syndrome ◽

Churg Strauss

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ANCA - Positive Churg Strauss Syndrome with Necrotizing Crescentic glomerulonephritis

JMS SKIMS ◽

10.33883/jms.v12i1.185 ◽

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)

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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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Primary vasculitides

Oxford Handbook of Rheumatology ◽

10.1093/med/9780199587186.003.0015 ◽

2011 ◽

pp. 405-430 ◽

Cited By ~ 1

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) (...

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

Journal of International Medical Research ◽

10.1177/03000605211048366 ◽

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.

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