II. Epidemiology

  1. Rare disease seen most often in men

III. Pathophysiology

  1. Similar to Classic Polyarteritis Nodosa
  2. Small Vessel, Granulomatous Vasculitis
    1. Multiple organ system involvement
    2. Interstitial Lung Disease

IV. Phases

  1. Phase 1: Prodromal (esp. Upper Respiratory)
    1. Severe Asthma
    2. Allergic Rhinitis
    3. Eczema
  2. Phase 2: Eosinophilic (esp. Lower Respiratory)
    1. Eosinophilic lung infiltrate
  3. Phase 3: Vasculitis (occurs within 3 years of onset)
    1. Granulomatous ANCA-associated Small Vessel Vasculitis

V. Signs: Interstitial Lung Disease

  1. Eosinophilic tissue infiltration

VI. Labs

VII. Complications (often cause of death)

  1. Coronary arteritis
  2. Myocarditis

IX. Management

X. References

  1. Calabrese in Ruddy (2001) Kelly's Rheum, p. 1179-81
  2. Eustace (1999) J Am Soc Nephrol 10(9):2048-55 [PubMed]

Images: Related links to external sites (from Bing)

Related Studies

Ontology: Churg-Strauss Syndrome (C0008728)

Definition (NCI) An autoimmune necrotizing vasculitis with the formation of granulomas. It is a pulmonary and systemic vasculitis associated with eosinophilia.
Definition (MSH) Widespread necrotizing angiitis with granulomas. Pulmonary involvement is frequent. Asthma or other respiratory infection may precede evidence of vasculitis. Eosinophilia and lung involvement differentiate this disease from POLYARTERITIS NODOSA.
Concepts Disease or Syndrome (T047)
MSH D015267
ICD10 M30.1
SnomedCT 195362002, 82275008
English Allergic granulomatosis, Allergic Angiitides, Allergic Angiitis, Allergic Granulomatous Angiitides, Allergic Granulomatous Angiitis, Angiitides, Allergic, Angiitides, Allergic Granulomatous, Angiitis, Allergic, Angiitis, Allergic Granulomatous, Churg Strauss Syndrome, Churg-Strauss Vasculitis, Granulomatous Angiitides, Allergic, Granulomatous Angiitis, Allergic, Syndrome, Churg-Strauss, Vasculitis, Churg Strauss, Vasculitis, Churg-Strauss, Allerg granulomatous angiitis, Polyarteritis with lung involvement [Churg-Strauss], Churg-Strauss Syndrome, allergic granulomatous angiitis (diagnosis), allergic granulomatous angiitis, allergic granulomatous angiitis (Churg-Strauss), Churg-Strauss vasculitis, Churg Strauss syndrome, Eosinophilic granulomatous vasculitis, Churg-Strauss Syndrome [Disease/Finding], churg-strauss vasculitis, strauss churg, allergic granulomatosis, strauss churg syndrome, allergic angiitis, churg strauss, allergic granulomatosis angiitis, churg strauss syndrome, churg-strauss syndrome, churg strauss vasculitis, Eosinophilic Granulomatous Vasculitis, Vasculitis, Eosinophilic Granulomatous, Allergic Angiitis and Granulomatosis, Granulomatous Vasculitis, Eosinophilic, Angiitis, Granulomatous Allergic, Allergic Granulomatous and Angiitis, Vasculitides, Eosinophilic Granulomatous, Granulomatous Vasculitides, Eosinophilic, Eosinophilic Granulomatous Vasculitides, Allergic Angiitis, Granulomatous, Angiitides, Granulomatous Allergic, Allergic Granulomatosis, Granulomatoses, Allergic, Granulomatous Allergic Angiitides, Allergic Angiitides, Granulomatous, Granulomatous Allergic Angiitis, Allergic Granulomatoses, Granulomatosis, Allergic, Churg-Strauss vasculitis (disorder), Allergic angiitis, Churg-Strauss syndrome, Allergic granulomatosis angiitis, Allergic granulomatous angiitis, CSS - Churg-Strauss syndrome, Allergic granulomatosis angiitis (disorder), allergic; angiitis, granulomatous, allergic; granulomatous angiitis, Churg-Strauss, angiitis; allergic granulomatous
Italian Angioite granulomatosa allergica, Sindrome di Churg Strauss, Granulomatosi allergica, Vasculite granulomatosa eosinofila, Angioite e granulomatosi allergica, Angioite allergica granulomatosa, Angioite allergica, Angioite granulomatoso-allergica, Vasculite di Churg-Strauss, Sindrome di Churg-Strauss
Dutch allergische granulomateuze vaatontsteking, syndroom van Churg-Strauss, eosinofiele granulomateuze vaatontsteking, allergische granulomatose, allergische angiïtis, allergisch; angiitis, granulomateus, allergisch; granulomateuze angiitis, angiitis; allergisch granulomateus, Polyarteriitis met longaandoening [Churg-Strauss], allergische granulomateuze angiïtis, Allergische angiitis, Allergische granulomateuze angiitis, Angiitis, allergische granulomateuze, Churg-Strauss-syndroom, Polyarteriitis met longaandoening, Syndroom, Churg-Strauss-, Vaculitis, Churg-Strauss-
French Syndrome de Churg Strauss, Vasculite granulomateuse à éosinophiles, Granulomatose allergique, Vascularite de Churg et Strauss, Vasculite de Churg et Strauss, Angéite de Churg-Strauss, Angéite allergique, Angéite granulomateuse allergique, Syndrome de Churg et Strauss, Syndrome de Churg-Strauss, SCS (Syndrome de Churg et Strauss)
German eosinophile granulomatoese Vaskulitis, allergische Granulomatose, Panarteriitis mit Lungenbeteiligung, allergische Angiitis, allergische granulomatoese Angiitis, Allergische Angiitis, Allergische Vaskulitis, Churg-Strauss-Syndrom, Vaskulitis, Churg-Strauss-, Allergische granulomatöse Angiitis, Allergische granulomatöse Vaskulitis, Angiitis, allergische granulomatöse, Vaskulitis, allergische granulomatöse
Portuguese Vasculite granulomatosa eosinófila, Granulomatose alérgica, Angeíte alérgica, Angeíte granulomatosa alérgica, Angiite Alérgica, Angiite Granulomatosa Alérgica, Síndrome de Churg-Strauss, Vasculite de Churg-Strauss
Spanish Granulomatosis alérgica, Vasculitis granulomatosa eosinofílica, Síndrome de Churg Strauss, angiítis granulomatosa alérgica, Angeítis alérgica, angeítis granulomatosa alérgica, síndrome de Churg - Strauss (trastorno), síndrome de Churg - Strauss, Angeítis granulomatosa alérgica, Angiitis Alérgica, Angiitis Granulomatosa Alérgica, Síndrome de Churg-Strauss, Vasculitis de Churg-Strauss
Japanese チャーグ・ストラウス症候群, アレルギー性肉芽腫症, 好酸球性肉芽腫性血管炎, アレルギーセイニクゲシュショウ, チャーグストラウスショウコウグン, コウサンキュウセイニクゲシュセイケッカンエン, アレルギーセイニクゲシュセイケッカンエン, チャーグ・ストラウス症候群, Churg-Strauss症候群, アレルギー性血管炎, アレルギーセイケッカンエン, Churg-Strauss脈管炎, アレルギー性肉芽腫性血管炎, チャーグ-ストラウス症候群, 血管炎-アレルギー性肉芽腫性, Churg Strauss症候群, チャウグ-シュトラウス症候群, チャーグ-ストラウス脈管炎, 脈管炎-チャーグ-ストラウス
Swedish Churg-Strauss syndrom
Czech Churgův-Straussové syndrom, angiitis allergica granulomatosa, Eozinofilní granulomatózní vaskulitida, Alergická granulomatózní angiitida, Alergická granulomatóza, alergická granulomatózní angiitida, syndrom Churga-Straussové, syndrom Churgův-Straussové, Alergická angiitida
Finnish Churg-Straussin oireyhtymä
Russian ALLERGICHESKII ANGIIT, ALLERGICHESKII GRANULOMATOZNYI ANGIIT, ANGIIT ALLERGICHESKII GRANULOMATOZNYI, VASKULIT CHERDZHA-STROSA, ЧЕРДЖА-СТРОСC СИНДРОМ, CHERDZHA-STROS SINDROM, ЭОЗИНОФИЛЬНЫЙ ГРАНУЛЕМАТОЗНЫЙ ВАСКУЛИТ, EOZINOFIL'NYI GRANULEMATOZNYI VASKULIT, CHERDZHA-STROSC SINDROM, ЧЕРДЖА-СТРОС СИНДРОМ, АЛЛЕРГИЧЕСКИЙ АНГИИТ, АЛЛЕРГИЧЕСКИЙ ГРАНУЛОМАТОЗНЫЙ АНГИИТ, АНГИИТ АЛЛЕРГИЧЕСКИЙ ГРАНУЛОМАТОЗНЫЙ, ВАСКУЛИТ ЧЕРДЖА-СТРОСА
Korean 폐 침습성 다발동맥염[처그-스트라우스]
Polish Zapalenie naczyń Churga i Strauss, Zespół Churga-Strauss, Zapalenie naczyń alergiczne ziarniniakowe
Hungarian Churg-Strauss-syndroma, Allergiás granulomás angiitis, Eosinophiliás granulomás vasculitis, Allergiás granulomatosis angiitis, Allergiás granulomatosis, Allergiás angiitis
Norwegian Polyarteritt med lungeaffeksjon, Allergisk granulomatøs angiitt, Allergisk granulomatøs vaskulitt, Allergisk vaskulitt, Churg-Strauss' syndrom