Vasculitis Syndromes Flashcards
Small vessels
Capillaries, arterioles, postcapillary venules
Medium vessels
Renal arteries, mesenteric vasculature, coronary arteries
Large vessels
Aorta and its proximal branches
ANCA association: cANCA
1) Wegener granulomatosis 2) Churg-Strauss
ANCA association: pANCA
1) Microscopic polyarteritis 2) Ulcerative colitis 3) Sclerosing cholangitis 4) Crohn disease %) Autoimmune hepatitis type 1
ANCA association: ANCA
Cystic fibrosis
Predominantly large vessel vasculitis
Takayasu arteritis
Predominantly medium vessel vasculitis
1) PAN 2) Kawasaki disease
Predominantly small vessel vasculitis
1) Granulomatosis with polyangiitis 2) Eosinophilic granulomatosis with polyangiitis 3) Microscopic polyangiitis 4) HSP 5) Isolated cutaneous luekocytoclastic vasculitis 6) Hypocomplementemic urticarial vasculitis
Granulomatosis with polyangiitis is aka
Wegener granulomatosis
Eosinophilic granulomatosis with polyangiitis is aka
Churg-Strauss syndrome
HSP affects more males vs females
Males
Approx 90% of HSP cases occur in what age group
Children, usually between 3 and 10y/o
T/F HSP in adults present with more severe and chronic complications
T
Common finding in biopsy of HSP is deposition of IgA particularly
IgA1
Hallmark of HSP
Palpable purpura
Skin lesions of HSP are usually of what distribution
Symmetric and in gravity-dependent areas or pressure points
Skin lesions of HSP typically last for how long
3-10 days and may recur up to 4 months after initial presentation
Arthritis and arthralgias occur in ___% of children with HSP
75
Arthritis in HSP is usually
1) Oligoarticular 2) Predilection for lower extremities 3) Does not lead to deformities 4) Usually resolves in 2 weeks
GI manifestations of HSP occur in up to ___% of patients with HSP
80
Renal involvement occurs in up to ___% of children with HSP
50
HSP ACR classification criteria
2 of the ff must be present 1) Palpable purpura 2) Age at onset less than or equal to 20 y/o 3) Bowel angina (postprandial abdominal pain, bloody diarrhea) 4) Biopsy demonstrating intramural granulocytes in small arterioles and/or venules
HSP EULAR criteria
1 or more must be present 1) Abdominal pain 2) Arthiris or arthralgia 3) Biopsy of affected tissue demonstrating predominant IgA deposition 4) Renal involvement (proteinuria >3g/24hr), hematuria or red cell casts
An isolated cutaneous leukocytoclastic vasculitis that affects infants <2 yr of age, that resembles HSP clinically
Acute hemorhhagic edema (AHE)
Distinguishes AHE from HSP
1) Younger age 2) Nature of the lesions 3) absence of other organ involvement 4) biopsy
T/F Platelet count in HSP is low
F, Normal
UTZ is done in patients with HSP and GI symptoms to look for
1) Bowel wall edema 2) Intussusception (rarely)
___ are most often used to treat significant GI involvement and joint pain in HSP
Steroids (Prednisone 1mkday for 1-2 weeks followed by taper)
T/F Prednisone in HSP does not alter overall prognosis nor prevent renal disease
T
Major long term complication of HSP
Renal disease
Renal disease in HSP can develop up to ___ after diagnosis
6 months, but rarely happens if initial UA is normal
Self-limited course of HSP usually lasts ___ on average
4 weeks
From 15-60% of children with HSP experience 1 or more recurrences, typically within ___ of diagnosis
4-6 mo
T/F HSP: With each relapse, symptoms are usually milder than at presentation
T
T/F HSP: Children with a more-severe initial course are at higher risk for relapse
T
T/F HSP: The long-term prognosis usually depends upon the severity and duration of gastrointestinal or renal involvement
T
Approximately ___% of those with HSP nephritis go on to have end-stage renal disease
8
AKA pulseless disease
Takayasu arteritis
Takayasu arteritis predominantly affects males vs females
Females
Complication of takayasu arteritis that is more common in United States, Western Europe, and Japan
Occlusive complications
Complication of takayasu arteritis that is more common in SEA and Africa
Aneurysms
MC aortic branches affected by takayasu arteritis
1) Subclavian 2) Renal 3) Carotid
___mmHg difference in systolic BP among 4 extremities is indicative of disease
> 10
Mainstay of therapy of Takayasu arteritis
Glucocorticoids, starting with high doses (Pred 1-2mkday)
T/F Cutaneous PAN is limited to the skin
T
Vessel involvement in PAN is usually
Segmental and tends to occur at vessel bifurcations
Vascular bed that is usually spared in PAN
Pulmonary vasculature
Gold standard diagnostic imaging study for PAN
Conventional arteriography
Conventional arteriography in PAN reveals
“Beads on a string” pattern (areas of aneurysmal dilation and segmental stenosis)
Mainstay of therapy in PAN
Oral (1-2mkday) and IV pulse (30mkday) prednisone
GPA and MPA involve what vascular beds
Respiratory tract and kidneys
A small vessel necrotizing granulomatous (allergic granulomatosis) vasculitis associated with a history of refractory asthma and peripheral eosinophilia
CSS
ANCA-associated vasculitis that can produce a saddle nose deformity
GPA
Small vessel vasculitis: IgA-dominant immune deposits
HSP
Small vessel vasculitis: Necortizing vasculitis
GPA, CSS, and MPA
Small vessel vasculitis: Granulomatous inflammation
GPA and CSS
Small cell vasculitis: Asthma and eosinophilia
CSS
Predominantly small vessel vasculitis, granulomatous
1) Wegener granulomatosis 2) Churg-Strauss
Predominantly small vessel vasculitis, nongranulomatous
1) Microscopic polyangitis 2) HSP 3) Isolated cutaneous leukocytoclastic vasculitis 4) Hypocomplementemic urticarial vasculitis
MC vasculitis of childhood
HSP
Characterized by leukocytoclastic vasculitis and IgA deposition in small vessels of the skin, joints, GIT, and kidneys
HSP
Takayasu arteritis is characterized by inflammation of the vessel wall starting at what layer
Vasa vasorum
Proposed classification criteria for pediatric Takayasu arteritis
Angiographic abnormalities of the aorta or its main branches AND at least one of the ff: 1) Decreased peripheral artery pulse and/or claudication of extremities 2) BBP diff between arms and legs of >10mmHg 3) bruits over the aorta and/or its major branches 4) Htn
T/F Radiographic assessment is essential to establish large vessel arterial involvement
T
Gold standard to establish large vessel arterial involvement in Takayasu arteritis
Conventional arteriography of the aorta and its major branches
