Disease Information for Churg-Strauss syndrome

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Clinical Manifestations
Signs & Symptoms
Bronchospasm signs
Dyspnea on exertion
Hemoptysis
Lung signs/abnormality
Rales
Wheezing
Nose blocked/stuffy
Runny nose/rhinorrhea
Constitutional symptoms
Clinical Presentation & Variations
Pulmonary Renal Syndromes
Presentation/Asthma Eosinophilia
Presentation/Treatment Failure Asthma
Demographics & Risk Factors
Established Disease Population
Patient/Allergic/atopic
Patient/Asthma
Laboratory Tests
Microbiology & Serology Findings
Microlab/Sputum/Eosinophiles on smear
Abnormal Lab Findings - Decreased
PLEURAL Glucose
Abnormal Lab Findings - Increased
Anti-neutrophilic/cytoplasm antibody (ANCA) (Lab)
Eosinophiles (LAB)
IGE/Immunoglobulin E (Lab)
P-ANCA antibodies (Lab)
Diagnostic Test Results
Pathology
PATH/ANCA Positive Vasculitis
BX/Gastric/Eosinophilic Gastritis
BX/Lung Abnormal (Biopsy)
BX/Lung biopsy/Granulomas in lung
BX/Lung biopsy/Necrotizing granulomas
BX/Lung biopsy/Vasculitic lung lesions
BX/Renal Glomerular Crescent Formation
BX/Renal Pauci-immune Glomerulonephritis
PATH/Eosinophilic infiltrate/tissue
PATH/Granulomatous tissue/specimen
X-RAY
Xray/Transient pulmonary infiltrates/Chest
Associated Diseases & Rule outs
Rule Outs
Ascariasis, pulmonary larval infestation
Silicosis
Wegeners granulomatosis
Associated Disease & Complications
Asthma
Churg Strauss pulmonary vasculitis
Eosinophilia Hypereosinophilia
Granulomatous lung disease
Mononeuritis, multiplex
Multi-radicular Polyneuropathy syndrome
Nasal obstruction
Vasculitis syndrome
Mononeuropathy
Asthma Children
Disease Mechanism & Classification
Class
CLASS/Vascular Small Vessel Disorder
CLASS/Lung Disorder (ex)
CLASS/Pulmonic (category)
Pathophysiology
Pathophysiology/Hypersensitivity Vasculitis
Pathophysiology/Small Vessel Vasculitis
Pathophysiology/Bronchospasm
Pathophysiology/Obstructive pulmonary disorder
Pathophysiology/Pulmonary vasculitis
Pathophysiology/ANCA Glomerulonephritis
Process
PROCESS/Allergy/collagen/autoimmune (category)
PROCESS/Eponymic (category)
PROCESS/Inflammatory/Granulomatous disease (ex)
PROCESS/Granulomatous Disease
Synonyms
Synonym
Allerg granulomatous angiitis, Allergic Angiitides, Allergic angiitis, allergic angiitis, granulomatous, Allergic granulomatosis, Allergic granulomatosis angiitis, Allergic granulomatosis angiitis (disorder), ALLERGIC GRANULOMATOSIS OF CHURG STRAUSS, Allergic Granulomatous Angiitides, Allergic Granulomatous Angiitis, Angiitides, Allergic, Angiitides, Allergic Granulomatous, angiitis allergic granulomatous, Angiitis, Allergic, ANGIITIS, ALLERGIC GRANULOMATOUS, CHURG STRAUSS, Churg Strauss syndrome, Churg Strauss vasculitis, CSS Churg Strauss syndrome, Granulomatous Angiitides, Allergic, Granulomatous Angiitis, Allergic, Polyarteritis with lung involvement (Churg Strauss), Syndrome, Churg Strauss, Vasculitis, Churg Strauss, Synonym/Allergic granulomatosis of lung, Synonym/Polyarteritis nodosa with pulmonary lesions, Synonym/Pulmonary variant/polyarteritis nodosa
Treatment
Drug Therapy - Indication
RX/Corticosteroid (Cortisone)
RX/Cyclophosphamide (Cytoxan)
RX/Inhaled/steroid
SX/Lung biopsy
Surgical Procedures or Treatments
SX/Biopsy
Definition

Widespread necrotizing angiitis with granulomas; Pulmonary involvement is usual; Asthma or other respiratory infection may precede evidence of vasculitis; Eosinophilia and lung involvement differentiate this disease from polyarteritis nodosa--------------------------------------------------Churg Strauss Syndrome; Allergic Angiitis and Granulomatosis; Allergic Granulomatosis; Allergic Granulomatosis and Angiitis; Churg-Strauss Vasculitis

Eosinophilic Granulomatous Vasculitis; Churg-Strauss Syndrome is a rare disorder that may affect multiple organ systems, particularly the lungs; The disorder is characterized by the formation and accumulation of an unusually large number of antibodies, abnormal clustering of certain white blood cells (eosinophilia), inflammation of blood vessels (vasculitis), and the development of inflammatory nodular lesions (granulomatosis);Many individuals with Churg-Strauss Syndrome have a history of allergy; In addition, asthma and other associated lung (pulmonary) abnormalities ( pulmonary infiltrates) often precede the development of the generalized (systemic) symptoms and findings seen in Churg-Strauss Syndrome by one or more years; Asthma, a chronic respiratory disorder, is characterized by inflammation and narrowing of the lungs’ airways, causing difficulties breathing (dyspnea), coughing, the production of a high-pitched whistling sound while breathing (wheezing), and/or other symptoms and findings; Nonspecific findings associated with Churg-Strauss Syndrome typically include flu-like symptoms, such as fever, a general feeling of weakness and fatigue (malaise), loss of appetite (anorexia), weight loss, and muscle pain (myalgia); Additional symptoms and findings may be variable, depending upon the specific organ systems involved; Without appropriate treatment, serious organ damage and potentially life-threatening complications may result; Although the exact cause of Churg-Strauss Syndrome is unknown, many researchers indicate that abnormal immunologic and autoimmune factors play an important role[NORD 2005]-----------------------------------------------------

The American College of Rheumatology (ACR) has proposed 6 criteria for diagnosis of CSS. The presence of 4 or more criteria yields a sensitivity of 85% and a specificity of 99.7%. These criteria are (1) asthma (wheezing, expiratory rhonchi), (2) eosinophilia of more than 10% in peripheral blood, (3) paranasal sinusitis, (4) pulmonary infiltrates (may be transient), (5) histological proof of vasculitis with extravascular eosinophils, and (6) mononeuritis multiplex or polyneuropathy.

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External Links Related to Churg-Strauss syndrome
Google
Wikipedia
Merck
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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