Vasculitis, Hyperreactivity, Veins and Lymphatics, Vascular Tumors Flashcards
Nonspecific signs of vasculitis
Fever, malaise, joint and muscle aches
Noninfectious vasculitis
Via immunologic (complex or autoantibody) injury
Infectious vasculitis
Pathogenic organisms invading the vessel wall
Treatment for NONINFECTOUS vasculitis
Immunosuppression
Large vessel vasculitis possibilities
- Giant cell (temporal) arteritis (>50)
- Takayasu arteritis (
Medium vessel vasculitis possibilities
- Polyarteritis nodosa (immune complexes)
- Kawasaki disease (anti-endothelial Ab’s)
Small vessel vasculitis possibilities (7)
- Microscopic polyangiitis (no asthma or granulomas)
- Wegener’s granulomatosis (granulomas, no asthma)
- Churg-Strauss (granulomas, asthma, eosinophilia)
- SLE (complex)
- HSP (IgA complex)
- Goodpasture’s (complex)
- Cryoglobulin vasculitis (complex)
Anti-proteinase-3
C-ANCA
Anti-myeloperoxidase
P-ANCA
T or F: ANCA levels correlate w/ disease severity
TRUE
MOA of ANCAs
Activate neutrophils that then release ROS
Giant cell (temporal) arteritis
- Vision loss and/or headache, non-specific symptoms
- T-cell inflammation of arteries in head (temporal, ophthalmic)
- Granulomatous inflammation w/ multinucleated giant cells
- PATCHY and FOCAL inflammation
- Scarring of media, thickening of intima
Takayasu arteritis
- Weak pulse and low BP in upper extremities
- Similar inflammation to temporal arteritis, but involving aortic arch and major branch vessels
- Younger age group (
Polyarteritis nodosa
- Young adult (could be any age though)
- Systemic vasculitis via immune complexes
- Renal > coronary > liver > GI tract
- LUNGS ARE SPARED
- Transmural necrotizing inflammation w/ fibrinoid necrosis
- 1/3 have Chronic Hepatitis B (HBsAg-Ab complexes)
Kawasaki’s disease
- Infants and small children ( aneurysm, rupture
- RISK OF ACUTE M.I.
- Anti-endothelial antibodies
Treatment of Kawasaki’s disease
Aspirin + IVIg (lower risk of coronary artery event)
Microscopic polyangiitis
- Necrotizing vasculitis of SMALL vessels
- Fibrinoid necrosis
- Renal glomeruli and lung capillaries
- P-ANCA
- Many apoptotic neutrophils
Churg-Strauss syndrome
- Necrotizing vasculitis of SMALL vessels
- ASTHMA, ALLERGIES, EOSINOPHILIA
- GRANULOMAS
- Otherwise similar inflammation to M.P. or PAN
Behcet disease
- Vasculitis of small/medium vessels
- ULCERS of ORAL CAVITY
- GENITAL ULCERS
- UVEITIS
- Neutrophilic, nonspecific inflammation
- HLA-B51 association
Granulomatosis w/ polyangiitis (Wegener’s)
- Males, 40’s
- Persistent pulmonary, URI, and renal disease
- Necrotizing vasculitis
- Granulomas of upper and lower respiratory tract
- Crescentic glomerulonephritis (RPGN)
- C-ANCA
- Treat w/ corticosteroids and cyclophosphamide
Thromboangiitis obliterans (Buerger’s disease)
- THROMBOSING vasculitis of small/medium vessels
- Tibial aa.
- Radial aa.
- Vascular insufficiency of DIGITS/extremities
- SMOKERS, young adults
- Adjacent veins and nerves can get inflammation too
- Chronic ulceration –> GANGRENE
Primary vs. Secondary Raynaud’s
Primary = COLD --> symmetric digits (benign) Secondary = part of arterial disease (SLE, scleroderma, Buerger's disease) (worsens with time)
2 causes of myocardial artery vasospasm
- Excess epinephrine (pheochromocytoma)
- Cocaine
Varicose veins
Risks?
- Dilation of veins via valvular incompetence
- Stasis, congestion, thrombus, edema, ischemia of skin
Portal hypertension can cause what 3 venous dilations?
- Esophageal varices (can fatally rupture)
- Hemorrhoids (rectal veins –> pain, bleeding, ulcerate)
- Caput medusa (periumbilical veins)
- Sitting for long period of time
- Venous congestion in deep leg
Risk?
DVT
Risk = pulmonary embolism
Trousseau sign
Migrating thrombophlebitis
- Often due to paraneoplastic hypercoaguability
- Usually MUCIN-producing ADENOcarcinomas of LUNG, OVARY, PANCREAS
Hemangioma
Common sites?
Local increased neoplastic blood vessel growth
- Skin, mucus membranes, liver
3 types of hemangioma (w/ descriptions)
- Capillary hemangioma (thin-walled capillaries tightly packed)
- Cavernous hemangioma (irregular, dilated vascular channels making lesion w/o distinct border; deep tissue)
- Pyogenic granuloma (LOBULAR capillary hemangioma) (NOT pyogenic, NOT a granuloma) (rapidly growing, oral mucosa)
Lymphangioma vs. hemangioma
Lymphangioma = NO RBCs
2 types of lymphangioma (w/ descriptions)
- Simple lymphangioma (head/neck and axilla)
- Cavernous lymphangioma (cystic hygroma) (neck or axilla of children, can be large) (TURNER’S SYNDROME)
Glomus tumor
Benign tumor via glomus bodies (distal fingers)
- PAINFUL
- SM origin
Bacillary angiomatosis
Vascular proliferation via Gram (-) Bartonella species
- IMMUNOCOMPROMISED
- Skin, localized, red papules, capillary proliferation
- Treat w/ macrolide antibiotics
Epithelioid hemangioendothelioma
Neoplastic endothelial cells resembling epithelium (cuboidal, plump)
Kaposi sarcoma - associated w/ what?
HHV8
4 types of Kaposi’s sarcoma (w/ descriptions)
- AIDS-associated KS (spread to LNs and viscera)
- Classic KS (no AIDS, older men of mediterranean descent, skin only)
- African KS (no AIDS,
Angiosarcoma
Malignant endothelial tumor
- Commonly in skin, soft tissue, breast, liver
- Older people
- Locally invasive and metastatic
- Relatively deadly
- CD31 marker
