249. Vasculitis

Question 1

Giant Cell Arteritis

type, Epidemiology, Path, Sx, Labs, Imaging, Biopsy, Tx)

Answer 1

Large Vessel Vasculitis
Most common form of systemic vasculitis in adults
F>M, avg age 73, WHITE
Path: inflammation on internal elastic lamina, multinucleated giant cells, granulomas, focal fibrinoid necrosis
Sx: Constitutional, new HEADACHE, POLYMYALGIA, jaw claudication, partical/complete vision loss
Labs: HIGH ESR/CRP
Imaging: Temporal Artery US
Biopsy: Lumen obliteration and internal elastic membrane destruction
Tx: high dose corticosteroids + taper, Anti-IL-6 (Tocilizumab)

Question 2

Takayasu Arteritis

Type, Epidemiology, Sx, Lab, Path, Imaging, Tx

Answer 2

Large Vessel Vasculitis (Aorta, Subclavian, Carotid)
Age < 40, F>M, Asian prevalence
Sx: constitutional, lightheaded, BRUITS, diminished pulses, BP difference in extremities
Lab: NORMAL
Path: Granulomatous panarteritis, intimal hyperplasia, fixed stenosis/occlusions, aneurysm
Imaging: CRUCIAL: noninvasive CTA/MRA/PET
Tx: high dose corticosteroids/taper, MTX/azathioprine to maintain remission, surgical bypass
EARLY RECOGNITION TO PREVENT MI/Ao Regurg

Question 3

Polyarteritis Nodosa
(type, Etiology, Epidemiology, Path, Sx, Tx)

Answer 3

Medium Vessel Vasculits
Idioipathic or 2/2 Hep B!
M>F, usually 40-60
Path: Transmural inflammation, no giant cells/granulomas, different phases of inflammation at different parts
Sx: Skin nodules, purpura, livedo reticularis, ulcers; renal disease, neuropathy, abd pain, orchitis, myalgia
Tx: Mild: Glucocorticoid (MTX if resistant); Moderate+: Glucocorticoid + Cyclophosphamide
TREAT HEP B if PRESENT

Question 4

Kawasaki Disease

Type, Epidemiology, Sx, Path, Labs, Tx

Answer 4

Medium Vessel Vasculitis
Young Children (0.5-5yrs) M>F, Asians
Sx: Livedo reticularis, strawberry tongue, lip cracking, pitting edema, erythematous rash
Path: predilection for coronaries, destruction of lumen endothelium, elastic lamina, and smooth muscle cells - leads to arterial wall dilation and aneurysm formation
Labs: anemia, leukocytosis, transaminitis
tx: ASA (high dose initially to prevent CVD)
IVIG - key to reduce coronary aneurysm
#1 CAUSE OF ACQUIRED HEART DISEASE IN KIDS

Question 5

Granulomatosis with Polyantiitis (wegener’s)

Type, Epidemiology, Assoc, Path, Sx

Answer 5

Small-medium vessel vasculitis
Age: bimodal (40-50 and 70+) Whites
Assoc with c-ANCA (PR3)
Path: necrotizing granulomas, pauci-immune
Sx: Pulm, Kidney, Arthritis, Gangrene Eye, Skin, Cardiac, GI problems
Tx: Steroids/taper, Rituximab/cyclophosphamide for severe disease

Question 6

Microscopic Polyangiitis

Type, Epidemiology, Path, Sx, Assoc

Answer 6

Small vessel vasculitis
Age: Middle Age
Path: necrotizing arteritis, pauci-immune, no granuloma
Sx: GN, pulm capillaritis, neruopathy, skin lesions, fevers, weight loss
assoc with p-ANCA (MPO)
Tx: Steroids/taper, Rituximab/cyclophosphamide for severe disease

Question 7

Eosinophilic GPA

Type, epidemiology, Assoc, Path, Sx

Answer 7

Small vessel vasculitis
M>F, age 35-45 y/o
Assoc with p-ANCA MPO or ANCA NEGATIVE
Eosinophilia/High IgE; adult onset asthma
Path: necrotizing vasculitis, eosinophilic infiltration, extravascular granuloma
Sx: 1. Prodrome - asthma, allergic rhinitis, nasal polyp
2. Eosinophilia
3. Vasculitis - end organ damage
Tx: Glucocorticoids, Cyclophosphamide
Mepolizumab (anti-IL-5 for allergic sx)

Question 8

Anti-GBM Disease (Goodpasture’s)

Type, Epidemiology, Etiology, Sx, Tx

Answer 8

IC small vessel vasculitis
Older children/adults
Idiopathic/following URI
Sx: acute renal failure, alveolar hemorrhage (can occur with ANCA vasculitis)
Tx: plasmapheresis, glucocorticoids, cyclophosphamide

Question 9

Cryoglobulinemia

type, Cryoglobulin definition, types, sx

Answer 9

IC small vessel vasculitis
Cryoglobulin: Ig’s or Ig/Complement that precipitate at tempts below 37
I. Monoclonal Ig’s (assoc with malignancy)
II. Mixed Mono/PolyClonal Ig’s (assoc with HEP C)
III. Polyclonal IgG/IgM
Hyperviscosity (digital ischemia, skin necrosis) vs. Vasculitis sx

Question 10

IgA Vasculitis

Type, Epidemiology, Etiology, Path, Sx, Tx

Answer 10

IC small vessel vasculitis
Children 3-15 y/o: Better prognosis
Few days after URI
Path: IgA/C3 in vessel, granulocytes in arteriole walls, necrotizing lesions
Sx: Rash - purpura (butt, lower legs, knees, elbows); Intestines (Ab pain, rash, V/D), Joint Pain/Myalgias, Kidney Involvement (hematuria)
Tx: supportive care, glucocorticoids - use to prevent CKD

Question 11

Bechets Syndrome

Type, Epidemiology, Sx, Tx

Answer 11

Mixed Vessel Vasculitis
30-49 y/o in Mediterranean, Mid East, far East
Sx: oral/genital ulcers, cutaneous lesions where blood is drawn, ocular involvement, arthralgias, CV/Neuro/Inner Ear Involvement
Tx: Organ Based (topical glucocorticoids, colchicine, Apremilast (PDE-4-i)), Systemic GCs/TNF-i’s/Azathioprine/Cyclophosphamide formore severe disease