Vasculitis

Disorder Vessels Pathology Presentation Test Tx Other

Arteries
Giant Cell Large to small aa. Granulomatous cell inflammation >50yo, w/ HA, ipsilateral ↑ ESR corticosteroids, anti Most common
(Temporal) – usually in (multinucleate giant cells) – nodular vision loss, pain w/ (sedimentation - inflammatories vasculitis in adults
Arteritis head/chest (esp. thickening – ↓ lumen (ischemia) – elastic chewing, polymyalgia rate)
temporal a.) lamina frag. rheumatica (m. pains) ↔ serum CK
Takayasu Arteritis Ao (arch) and ↓ lumen – granulomatous – patchy Japanese woman <40 w/ Aortogram w/ “Pulseless Dz”
branches necrosis of media – dense circumferential absent pulse – extremity dye Auto-immune
collagenous scarring numbness/coldness/ cause suspected
claudication, pulm HTN, HLA-DR assoc.
vision/neuro problems
Polyarteritis Medium, small Infarction/ischemia of organs – Young adult w/ Angiogram Corticosteroids, NO giant cells
Nodosa (PAN) aa. – renal and transmural inflammation (NOT constitutional sx (fever, cyclophosphamide All stages present
visceral (ht, liver, Granulomatous) – fibrinoid necrosis weight loss, malaise, m. at same time
GI) (thrombosis of vessels) – late = fibrous pain) or GI bleeding, renal 30% HBV
thickening into adventitia failure/hematuria (w/o No ANCA
glomerulonephritis)
Kawasaki Dz Medium aa Necrosis and inflammation of entire wall Japanese child (esp. <4, ECG ASA #1 cause ped MI
(Mucocutaneous esp. Cornary aa. (acute phase) – PAN-like but less fibroid boys>girls) w/ acute sx of IVIg Anti-EC, smooth
Lymph Node or hands/feet necrosis – post-inflammation thrombi and fever, erythema, edema, Ø corticosteroids m. Abs?
Syndrome) aneurysms desquamation of hands/feet, Genetic + trigger
cervical lymphadenoapthy
Microscopic Small Necrotizing vasculitis (esp. Palpable purpura, mucus P-ANCA+ Immune rxn to
Polyangiitis glomerulonephritis) – leukocytocystic – memb. lesions, hemoptysis drug, infection,
(polyarteritis) transmural infiltrate – less fibrinoid arthralgia, abdomen pain tumor Ag
Same stage in all
Wegener’s Medium, small Triad: (1) Acute necrotizing granulomas >40 (men>women) bilateral C-ANCA+ Immuno- Saddle nose
Granulomatosis (esp lung) in URT (2) necrotizing vasculitis in nodular and cavitary pulm. suppressive tx Hypersensitivity
lungs/URT (3) focal necrotizing Infiltrates, sinusitis, renal to inhaled
glomerulonephritis (crescent) dz, nasopharyngeal ulcers infectious/enviro
agent?
Thromboangitis Medium and Digital vessel thrombosis (ischemia in Young to middle-aged man Stop smoking Ab to something
Obliterans small aa. in fingers/toes) – luminal thrombi w/ micro who smokes, pain in fingers IV iloprost (PG in smoke
(Buerger’s Dz) extremities abscesses- inflammation extends to (maybe ulcerations or analogue)
(radial, tibial) adjacent vv. and nn. gangrene)
Raynaud’s Medium (esp Cold-induced vasoconstriction Young (female>male) Avoid cold Familial, benign
Phenomenon digits, nose, ears) Pallor/cyanosis of acral Ca+2 channel usually
parts, (white→blue→red) blockers Chronic – atrophy
of skin, subcu, m.
2° Raynaud’s Medium (acral) Vasospasm d/t dz (SLE, Buerger’s), Pallor/cyanosis of acral Avoid cold
Systemic sclerosis, CREST, parts in adult >30, Ca+2 channel
ulceration, gangrene blockers
 Vasculitis

Disorder Vessels Pathology Presentation Complications Other

Veins
Varicose Veins Superficial Veins Abnormally dilated, tortuous vv. d/t ↑ >50yo, obesity, prolonged standing, Venous stasis, edema, Familial tendency
pressure – varibly thinned and scarred post-pregnancy woman pain, thrombosis, poor
(thickened) – phlebosclerosis (spotty wound healing (ulcers),
calcification) stasis dermatitis
Esophageal Esophageal vv. Portal HTN, chirrosis Varicose vv. in esophagus Massive hemorrhage Often fatal
Varices
Hemorrhoids Portal HTN, pregnancy Vericose veins in AJ (anal junction) Thrombosis and ulcers
Venous Deep leg vv, portal Virchow’s Triad: stasis, endothelial injury, Pain in leg or flexing foot, edema Pulmonary emboli
Thrombosis v., dural sinuses hypercoagulability
(DVTs)
Thrombophlebitis Superficial vv. Infection (Staph Aureus) often d/t IV
insertion/infiltration
Superior Vena SVC Extrinsic compression of SVC (lung apex Swelling, blue discoloration of face
Cava Syndrome small cell ca)
Lymphangitis Lymph vessels Incomplete basement membranes predispose Red streak along lymphatic course GAS most common
to infection/tumor invasion
Lymphedema Lymph vessels Occlusion of lymph drainage d/t tumor, Swelling distally
surgical removal of nodes, radiation fibrosis,
filariasis, post-inflamm scarring
Vascular Tumors
Benign Hemangiomas
Capillary Skin, oral mucosa Non-encapsulated close packed capillaries – “Strawberry” on juvenile, flat to
blood-filled – little stroma – may be raised
thrombosed
Cavernous Deep structures Large blood-filled spaces, sharply defined not Grossly red-blue, soft and spongy, Von Hippel-Lindau
encapsulated, scant stroma, can be 1-2cm (in brain, eye,
thrombosed or calcifications pancreas, liver)
Pyogenic Skin of oral Polypoid, rapidly growing, on stalk – post “Lobular capillary”
mucosa trauma – granulomatous histologically-
inflammation – exuberant healing process
Glomus Small vessels of Modified smooth m. cells of glomus body – Painful
fingertips nests of glomus cells around vessels Under fingernail
Cavernous Large, neck, axilla No blood in vascular lumens – On neck in Cystic Hygroma
Lymphangioma Turner’s Syndrome (XO) – Non-encapsulated
Bacillary Skin, bone, brain, Opportunistic infection (Bartonella family Red papules, neutrophils and Cat-Scratch fever
Angiomatosis other organs bacteria) – proliferation of capillaries w/ bacteria, silver stain of bacilli
endothelial cells
 Vasculitis
Disorder Vessels Pathology Presentation Complications Other
Intermediate and Malignant
Kaposi Sarcoma Skin Patch: dilated irregular vessels, inflamm like Grossly patch→plaque→ nodules Local excision w/
granulomatous tissue pink to purple good results – more
Plaque: Dilated jagged vascular channels w/ lesions= radiation,
plump spindle cells w/ chronic inflamm cells chemo, antivirals
Nodule: same but with mitoses, hemosiderin
and hemorrhage
Herpesvirus 8 (KSHV) – necessary and
sufficient – sexually transmitted?
Chronic Older men, Red-purple plaques Ashkenazi Jews
Lymph- Lymphadenopathy, aggressive Young Africans HIV+ and HIV-
adenopathic
Transplant Lymph nodes, Aggressive
mucosa, viscera Usually fatal
AIDS All over Death d/t infection, not KS Most common
AIDS cancer

Angiosarcoma Endothelial Environmental – PVC, Arsenic, Thorotrast – Older adults, asymptomatic red Mesenchymal
Radiation induced – chronic lymphedema – nodules anywhere on body tumor
local invasions and metastases (advanced = lg. gray white fleshy Poor survival
masses w/ central necrosis and
hemorrhage)

MOI
1. Immune complex deposition (Ag-Ab deposition in wall or formed in situ)
- ANCA – anitneutrophil cytoplasmic Ab
• c-ANCA – cytoplasmic staining against PR3 – i.e. Wegener’s granulomatosis
• p-ANCA – perinuclear staining against myeloperoxidase – i.e. Microscopic polyarteritis, Churg-Strauss
2. Direct Ab mediated
- Anti-endothelial cell Ab – i.e. SLE, Kawasaki
- Anti-basement membrane Ab – i.e. Goodpasture’s
3. Direct Infection
- Ricksettial (Rocky Mt. Spotted Fever)
- Viral (HBV, Herpes Zoster)
- Fungal (Aspergillus, Mucomycosis)
- Spirochete (Syphilis)
- Bacterial (Neisseria)
4. Unknown – Takayasu, Giant Cell, PAN