| What is vasculitis? | Presence of inflammatory leukocytes in vessel walls with reactive damage to mural structures.
Muscular arteries may develop focal or segmental lesions and these may be life threatening,
Inflammation lead to aneurysm, vessel rupture or segmental lesions causing stenosis, occlusion with distal infarction myabe.
Affected vessels vary in size, type, location according to specific types of vasculitis, large or medium or small vessels.
Exact pathobiology is unknown and multifacorial, sometimes fatal diseases that require prompt recognition and therapy, may get symptoms either isolated or multisystem. |
| What are large vessel vasculitis? | Takayasu arteritis /Pulseless disease (aorta and its major branches, localized to a portion of the affected vessel, pan-aortitis, <30 year of age, many types, I affected vessels are branches of aorta, IIa are ascending aorta, arch and branches, IIb ascending aorta, branches, arch and thoracic descending aorta, III thoracic and abdomina aorta, IV abdominal and renal aorta, V all together)
Giant Cell Arteritis/ Horton's disease/ Temporal Arteritis (aorta and its main branches predilection of carotid including superficial temporal arteries, age >50 years, high inflammatory markers, constitutional symptoms, headache, jaw claudication, eye diploplia, loss of vision, neuropathy, polymyalgia rheumatica)
Other forms (no specific name, idiopathic isolated aortitis, part of another form of vasculitis or systemic inflammatory condition like Cogans syndrome/relapsing polychondritis) |
| What are medium vessel vasculitis? | Polyarteritis nodosa (systemic necrotizing vasculitis, mononeuritis multiplex 60%, HepB, medium and small sized arteries)
Kawasaki (medium and small sized arteries but aorta and large arteries maybe involved, children, associated with mucucutaneous lymph node syndrome, coronary arteries may also be involved) |
| What are small vessel vasculitis autoimmune vasculitis? | ANCA-associated vasculitis (necortizing vasculitis, no immune complex deposition, specific for myeloperoxidase or proteinase 3 ANCA, cases of ANCA negative may occur in eosinophilic granulomatosis with polyangiitis, can occur only in a single organ especially renal limited AAV)
Microscopic polyangiitis (necortizing vasculitis, glomerulonephritis (rapid progression), pulmonary capillaritis, ANCA + >90%, negative angiogram)
Granulomatousis with Polyangiitis/ Wegners (necoritzing vasculitis, small-medium sized vessels, granulomatous inflammation of upper and lower respiratory tracts and glumoerulonephritis, ANCA +>80%, overlaps with MPA, anti-MPO/PR3)
Eosinophilic granulomatosis with polyangiitis/Chrug-Stauss (eosinophilic rich necrotizing vasculitis, chronic rhinosinusitis, asthma, blood eisonphilia, ANCA+>40%, less common glomerulonephritis) |
| What are immune complex small-vessel vasculitis? | Antiglomerular basement membrane disease (affect glomerular capillaries with basement membrane, lung involvement causes pulmonary hemorrhage, renal causes glomerulonephritis with necorsis)
Cryoglobinemic vasculitis (presence of cryoglubulin serum protein precipitate in the cold dissolve in warm, most often due to HepC, deposit in arteries, capillaries and venules usually skin, glomeruli, peripheral nerves)
IgA vasculitis/Henoch-Schonlein purpura (tissue deposit IgA complex in small vessels, typically skin, GI and arthritis)
Hypocomplementemic urticarial vasculitis/anti-C1q vasculitis (with urticaria and hypocomplementemia, and predominantly affects small vessels. Glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation may also be observed) |
| What are variable vessel vasculitis? | Behjet Syndrome (rare chronic inflammatory multisystem disorder, affect any artery or vein, Japan, Middleeast, M:F 2:1, genetic disposition (HLAB51, familial hx +) characterized by (skin and mucus membrane, eye panuveitis, CNS, thrombosis and aneurysms, GI)
Cogan's syndrome ( Eye: ocular inflammatory lesions, including interstitial keratitis, uveitis, and episcleritis as well as inner ear disease (eg, sensorineural hearing loss and vestibular dysfunction), ear hearing loss, vestibular symptoms) |
| What are criteria of diagnosis of Behjet disease? | . |
| What is single organ vasculitis? | • The involved organ and vessel type should be included in the name (eg, primary central nervous system vasculitis
[CNSV], cutaneous leukocytoclastic vasculitis, isolated aortitis)
• Some patients initially diagnosed with single-organ vasculitis may develop other disease manifestations, warranting
reevaluation for another systemic vasculitis (eg, cutaneous arteritis later becoming polyarteritis nodosa). |
| What is primary CNS vasculitis? | Primary CNSV, or primary angiitis of the CNS (PACNS), refers to vasculitis affecting the medium and small blood vessels of the brain, spinal cord, and the meninges, without systemic (non-brain) involvement |
| What is vasculitis associated with systemic disease? | • Subsets of patients with systemic lupus erythematosus, rheumatoid arthritis, relapsing polychondritis, and other
systemic rheumatic diseases may develop an associated vasculitis
• Most frequently involves small muscular arteries, arterioles, and venules |
| What is vasculitis associated with probable etiology? | • Examples include hepatitis C virus-associated cryoglobulinemic vasculitis, hepatitis B virus-associated polyarteritis
nodosa, and hydralazine-associated ANCA-associated vasculitis
• Hematologic and solid organ neoplasms as well as clonal B cell lymphoproliferative disorders can also be associated
with vasculitis. |
| How is diagnostic approach to vasculitis? | • patterns of organ injury
• the size of the vessels affected
• histopathological features
• characteristic findings on diagnostic imaging
hx (eye scleritis, nose, skin, CNS, PNS, lungs, kidneys, abdomen, drugs, rheumatic disease, infection) |
| How is physical exam of vasculitis? | identify potential sites of vasculitis, determine the extent of vascular lesions, the distribution of affected organs, and the presence of additional disease processes.
see skin (palpable purpura ->cutaneous leukocytoclastic vasculitis, small vessel vasculitis/polyarteritis nodosa, livedo reticularis (lace like purple discoloration of skin)
Vascular exam (absent, diminished or tender pulses)
Neuro exam (diminished sensation to touch, motor weakness, sensory/motor neuropathy) |
| What are lab tests for vasculitis? | CBC, creatine, LFT, ESR, CRP, viral hep serology, cryoglobulin, urinanalysis, blood culture, ANA, complements, ANCA (PR3/MPO)
HRCT, electromyography, lumbar puncture |
| What are imagings of vasculitis? | MRI, MR angiogram, CT angio, PET, vascular US GCA, conventiona angiography, no angiographic abnormality is specific for vasculitis |
| How is biopsy for vasculitis? | GCA, Skin, Renal important, cannot be used for aortic arch and primary branches |
