II. Epidemiology

  1. Most common ANCA-associated Small Vessel Vasculitis
  2. Age of onset peaks at 40 to 60 years
  3. Gender: Men more commonly affected

III. Symptoms

  1. Fever
  2. Arthralgias and Myalgias
  3. Cough (Hemoptysis may be present)

IV. Signs

  1. Renal involvement most common (Glomerulonephritis)
  2. Palpable Purpura
  3. Lung disease (pulmonary Hemorrhage)

V. Differential Diagnosis

  1. Polyarteritis Nodosa
  2. ANCA-associated Small Vessel Vasculitis
    1. Especially pulmonary-renal syndromes
    2. Wegener's Granulomatosis
    3. Systemic Lupus Erythematosus
    4. Anti-glomerular basement membrane disease

VI. Labs

  1. Antineutrophil Cytoplasmic Antibody (ANCA)
    1. pANCA (MPO-ANCA) positive in 60-85% of cases
    2. cANCA (PR3-ANCA) positive in 40% of cases

VII. Diagnostics

  1. Biopsy shows minimal to no immune vascular deposits

VIII. Management

IX. References

  1. Calabrese in Ruddy (2001) Kelly's Rheum, p. 1176-8
  2. Schoen in Cotran (1999) Robbins Pathology, p. 521-2

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Ontology: Vasculitis, Leukocytoclastic, Cutaneous (C0151436)

Definition (MSH) Disorder characterized by a vasculitic syndrome associated with exposure to an antigen such as a drug, infectious agent, or other foreign or endogenous substance. Its pathophysiology includes immune complex deposition and a wide range of skin lesions. Hypersensitivity or allergy is present in some but not all cases.
Concepts Disease or Syndrome (T047)
MSH D018366 , D014658
ICD9 446.20, 446.2
ICD10 M31.0 , D69.0
SnomedCT 195352009, 195350001, 60555002
English Hypersensitivity Vasculitis, Angiitides, Hypersensitivity, Angiitis, Hypersensitivity, Hypersensitivity Angiitides, Hypersensitivity Angiitis, Hypersensitivity Vasculitides, VASCULITIS ALLERGIC, Vasculitides, Hypersensitivity, Hypersensitivity angiitis NOS, VASCULITIS NONSPECIFIC ALLERGIC <HYPERSENSITIVITY VASCULITIS>, hypersensitivity vasculitis (diagnosis), hypersensitivity angiitis, hypersensitivity vasculitis, hypersensitivity angiitis (diagnosis), VASCULITIS NONSPECIFIC ALLERGIC HYPERSENSITIVITY VASCULITIS, Hypersensit angiitis NOS, Allergic Cutaneous Vasculitis, Cutaneous Leukocytoclastic Angiitis, Leukocytoclastic Angiitis, Cutaneous, Vasculitis, Cutaneous Leukocytoclastic, Allergic Cutaneous Angiitides, Cutaneous Allergic Vasculitides, Allergic Vasculitides, Cutaneous, Allergic Vasculitis, Cutaneous, Angiitides, Cutaneous Leukocytoclastic, Leukocytoclastic Vasculitis, Cutaneous, Angiitis, Cutaneous Leukocytoclastic, Cutaneous Leukocytoclastic Vasculitis, Vasculitides, Cutaneous Leukocytoclastic, Cutaneous Vasculitis, Allergic, Cutaneous Angiitis, Allergic, Angiitis, Allergic Cutaneous, Cutaneous Leukocytoclastic Vasculitides, Vasculitis, Allergic Cutaneous, Allergic Cutaneous Vasculitides, Cutaneous Vasculitides, Allergic, Allergic Cutaneous Angiitis, Vasculitis, Hypersensitivity, Cutaneous Leukocytoclastic Angiitides, Vasculitides, Allergic Cutaneous, Cutaneous Allergic Vasculitis, Vasculitides, Cutaneous Allergic, Vasculitis, Cutaneous Allergic, Leukocytoclastic Angiitides, Cutaneous, Leukocytoclastic Vasculitides, Cutaneous, Angiitides, Allergic Cutaneous, Cutaneous Angiitides, Allergic, Vasculitis, Leukocytoclastic, Cutaneous [Disease/Finding], allergic vasculitis, leukocytoclastic angiitis, leukocytoclastic vasculitis, leucocytoclastic vasculitis, vasculitis leukocytoclastic, Vasculitis, Leukocytoclastic, Cutaneous, Hypersensitivity angiitis NOS (disorder), Allergic cutaneous angiitis, Vasculitis allergic, Hypersensitivity angiitis, unspecified, Hypersensitivity angiitis, Hypersensitivity vasculitis, Allergic vasculitis, Leukocytoclastic vasculitis, Leucocytoclastic angiitis, Leucocytoclastic vasculitis, Maladie trisymptome, Trisymptome, Hypersensitivity angiitis (disorder), Leukocytoclastic angiitis, hypersensitivity; angiitis, allergic; vasculitis, angiitis; hypersensitivity, vasculitis; allergic, Vasculitis, allergic
French ANGEITE ALLERGIQUE, Angéite allergique, Angéite d'hypersensibilité, Vascularite leucocytoclasique cutanée, Vascularite allergique cutanée, Vascularite allergique, Angéite cutanée allergique, Angéite d'hypersensibilité, non précisée, Vasculite d'hypersensibilité, Angéite leucocytoclasique, Vascularite leucocytoclasique, Vascularite d'hypersensibilité, Vasculite allergique
Czech angiitida z hypersenzitivity, vaskulitida leukocytoklastická kožní, angiitida leukocytoklastická kožní, vaskulitida alergická, Alergický zánět cév, Hypersenzitivní angiitida, blíže neurčená, Hypersenzitivní vaskulitida, Alergická kožní angiitida, Hypersenzitivní angiitida, Alergická vaskulitida
Finnish Allerginen verisuonitulehdus, Ihon allerginen vaskuliitti
Russian VASKULIT ALLERGICHESKII, ANGIIT, GIPERSENSIBILIZATSIIA, VASKULIT, GIPERSENSIBILIZATSIIA, АЛЛЕРГИЧЕСКИЙ КОЖНЫЙ АНГИИТ, ALLERGICHESKII KOZHNYI ANGIIT, АЛЛЕРГИЧЕСКИЙ КОЖНЫЙ ВАСКУЛИТ, ВАСКУЛИТ АЛЛЕРГИЧЕСКИЙ КОЖНЫЙ, VASKULIT LEIKOTSITOKLASTICHESKII KOZHNYI, ВАСКУЛИТ АЛЛЕРГИЧЕСКИЙ, ALLERGICHESKII KOZHNYI VASKULIT, АНГИИТ АЛЛЕРГИЧЕСКИЙ КОЖНЫЙ, ANGIIT ALLERGICHESKII KOZHNYI, VASKULIT ALLERGICHESKII KOZHNYI, ВАСКУЛИТ ЛЕЙКОЦИТОКЛАСТИЧЕСКИЙ КОЖНЫЙ, АНГИИТ, ГИПЕРСЕНСИБИЛИЗАЦИЯ, ВАСКУЛИТ, ГИПЕРСЕНСИБИЛИЗАЦИЯ
German Hypersensitivitaetsangiitis, VASKULITIS ALLERGISCH, Angiitis, Hypersensitivitaets-, Vaskulitis, Hypersensitivitäts-, Vaskulitis, leukozytoklastische, kutane, anaphylaktische Angiitis, Hypersensitivitaetsvaskulitis, allergische Vaskulitis, allergische kutane Angiitis, Hypersensitivitaetsangiitis, unspezifisch, Vaskulitis allergisch, Angiitis, leukozytoklastische, Leukozytoklastische Vaskulitis, Vaskulitis, leukozytoklastische, Angiitis, Hypersensitivitäts-, Hypersensitivitätsangiitis, Hypersensitivitätsvaskulitis
Portuguese VASCULITE ALERGICA, Vasculite de Hipersensibilidade, Vasculite Leucocitoclástica Cutânea, Angeíte por hipersensibilidade, Angeíte cutânea alérgica, Angeíte por hipersensibilidade NE, Vasculite por hipersensibilidade, Vasculite alérgica, Angiite de Hipersensibilidade
Spanish VASCULITIS ALERGICA, Vasculitis Leucocitoclástica Cutánea, Vasculitis por Hipersensibilidad, angiítis por hipersensibilidad, angeítis por hipersensibilidad, SAI, angeítis por hipersensibilidad, SAI (trastorno), Angeítis cutánea alérgica, Angeítis por hipersensibilidad, Vasculitis por hipersensibilidad, Vasculitis alérgica, Angeítis por hipersensibilidad no especificada, angeítis por hipersensibilidad, vasculitis alérgica, vasculitis leucocitoclástica (trastorno), vasculitis leucocitoclástica, vasculitis por hipersensibilidad, Angitis por Hipersensibilidad
Korean 과민성 혈관염
Italian Vasculite leucocitoclastica cutanea, Vasculite allergica, Angioite da ipersensibilizzazione non specificata, Angioite da ipersensibilizzazione, Angioite cutanea allergica, Vasculite da ipersensibilità
Swedish Vaskulit, allergisk, kutan
Polish Alergiczne zapalenie naczyń, Zapalenie naczyń skóry z nadwrażliwości, Zapalenie naczyń na tle nadwrażliwości, Zapalenie małych naczyń skóry, Zapalenie naczyń leukocytoklastyczne, Leukocytoklastyczne zapalenie naczyń
Japanese 血管炎-過敏性, 血管炎-皮膚アレルギー性, 皮膚白血球破砕性血管炎, アレルギー性血管炎, アレルギー性皮膚血管炎, 皮膚アレルギー性血管炎, アレルギー性皮膚脈管炎, 血管炎-アレルギー性皮膚, 血管炎-皮膚白血球破砕性, 過敏性脈管炎、詳細不明, アレルギーセイケッカンエン, アレルギーセイヒフケッカンエン, カビンセイケッカンエン, カビンセイミャクカンエンショウサイフメイ, 白血球破砕性脈管炎, 白血球破砕性血管炎, 過敏性脈管炎, 過敏性血管炎, 脈管炎-白血球破砕性, 脈管炎-過敏性, 血管炎-アレルギー性
Norwegian Kutan allergisk vaskulitt, Hypersensitiv angiitt, Hypersensitiv vaskulitt, Kutan leukocytoklastisk vaskulitt, Kutan allergisk angiitt
Dutch overgevoeligheidsangiïtis, niet-gespecificeerd, allergische vasculitis, overgevoeligheidsangiïtis, overgevoeligheidsvasculits, allergische cutane angiïtis, allergisch; vasculitis, angiitis; overgevoeligheid, overgevoeligheid; angiitis, vasculitis; allergisch, Angiitis, leukocytoclastische, Angiitis, overgevoeligheids-, Overgevoeligheidsangiitis, Overgevoeligheidsvasculitis, Vasculitis, leukocytoclastische, Vasculitis, overgevoeligheids-
Hungarian Allergiás vasculitis, Allergiás cutan angiitis, Hypersensitivitas angiitis, nem meghatározott, Hypersensitivitas vasculitis, Vasculitis, allergiás, Hypersensitivitas angiitis

Ontology: Microscopic Polyangiitis (C0343192)

Definition (MSH) A primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls.
Concepts Disease or Syndrome (T047)
MSH D055953
ICD10 M31.7
SnomedCT 239928004
English Microscop polyarteritis nodosa, microscopic polyarteritis nodosa, microscopic polyarteritis nodosa (diagnosis), Polyangiitides, Microscopic, Microscopic Polyangiitides, Polyangiitis, Microscopic, Microscopic Polyangiitis, Microscopic Polyangiitis [Disease/Finding], microscopic polyangiitis, polyarteritis nodosa microscopic, Microscopic polyangiitis, Microscopic polyarteritis nodosa, Microscopic polyarteritis nodosa (disorder)
Japanese 顕微鏡的多発血管炎, ケンビキョウテキタハツケッカンエン
Czech Mikroskopická polyangiitida, mikroskopická polyangiitida
Korean 현미경적 다발혈관염
French Polyangéite microscopique
German Mikroskopische Polyangiitis, mikroskopische Polyangiitis
Italian Poliangite microscopica
Portuguese Poliangiite Microscópica, Poliangite microscópica
Spanish Poliangitis Microscópica, poliangiítis microscópica (trastorno), poliangiítis microscópica, poliarteritis nudosa microscópica (trastorno), poliarteritis nudosa microscópica, Poliangitis microscópica
Russian POLIANGIIT MIKROSKOPICHESKII, ПОЛИАНГИИТ МИКРОСКОПИЧЕСКИЙ, МИКРОСКОПИЧЕСКИЙ ПОЛИАНГИИТ, MIKROSKOPICHESKII POLIANGIIT
Swedish Mikroskopisk polyangiit
Polish Zapalenie małych naczyń
Hungarian Mikroszkópos polyangiitis
Norwegian Mikroskopisk polyangiitt
Dutch microscopische polyangiïtis

Ontology: Microscopic Polyarteritis (C2347126)

Definition (NCI) A systemic necrotizing vasculitis that typically affects the small and medium-sized muscular arteries. In some cases, however, microscopic vessels are also affected (e.g., in the kidneys), a condition that has been called microscopic polyarteritis or polyangiitis; this disorder is felt to be more closely associated with Wegener's granulomatosis than to classic polyarteritis nodosa.
Concepts Disease or Syndrome (T047)
ICD10 M31.7
English MPA, Microscopic Polyarteritis, Microscopic polyarteritis