Vasculitis

Disorder
Arteries Giant Cell (Temporal) Arteritis Takayasu Arteritis

Vessels
Large to small aa. – usually in head/chest (esp. temporal a.) Ao (arch) and branches

Pathology
Granulomatous cell inflammation (multinucleate giant cells) – nodular thickening – ↓ lumen (ischemia) – elastic lamina frag. ↓ lumen – granulomatous – patchy necrosis of media – dense circumferential collagenous scarring Infarction/ischemia of organs – transmural inflammation (NOT Granulomatous) – fibrinoid necrosis (thrombosis of vessels) – late = fibrous thickening into adventitia Necrosis and inflammation of entire wall (acute phase) – PAN-like but less fibroid necrosis – post-inflammation thrombi and aneurysms Necrotizing vasculitis (esp. glomerulonephritis) – leukocytocystic – transmural infiltrate – less fibrinoid Triad: (1) Acute necrotizing granulomas in URT (2) necrotizing vasculitis in lungs/URT (3) focal necrotizing glomerulonephritis (crescent) Digital vessel thrombosis (ischemia in fingers/toes) – luminal thrombi w/ micro abscesses- inflammation extends to adjacent vv. and nn. Cold-induced vasoconstriction

Presentation
>50yo, w/ HA, ipsilateral vision loss, pain w/ chewing, polymyalgia rheumatica (m. pains) Japanese woman <40 w/ absent pulse – extremity numbness/coldness/ claudication, pulm HTN, vision/neuro problems Young adult w/ constitutional sx (fever, weight loss, malaise, m. pain) or GI bleeding, renal failure/hematuria (w/o glomerulonephritis) Japanese child (esp. <4, boys>girls) w/ acute sx of fever, erythema, edema, desquamation of hands/feet, cervical lymphadenoapthy Palpable purpura, mucus memb. lesions, hemoptysis arthralgia, abdomen pain >40 (men>women) bilateral nodular and cavitary pulm. Infiltrates, sinusitis, renal dz, nasopharyngeal ulcers Young to middle-aged man who smokes, pain in fingers (maybe ulcerations or gangrene) Young (female>male) Pallor/cyanosis of acral parts, (white→blue→red) Pallor/cyanosis of acral parts in adult >30, ulceration, gangrene

Test
↑ ESR (sedimentation rate) ↔ serum CK Aortogram w/ dye

Tx
corticosteroids, anti - inflammatories

Other
Most common vasculitis in adults

“Pulseless Dz” Auto-immune cause suspected HLA-DR assoc. Corticosteroids, cyclophosphamide NO giant cells All stages present at same time 30% HBV No ANCA #1 cause ped MI Anti-EC, smooth m. Abs? Genetic + trigger Immune rxn to drug, infection, tumor Ag Same stage in all Saddle nose Hypersensitivity to inhaled infectious/enviro agent? Ab to something in smoke Familial, benign usually Chronic – atrophy of skin, subcu, m.

Polyarteritis Nodosa (PAN)

Medium, small aa. – renal and visceral (ht, liver, GI) Medium aa esp. Cornary aa. or hands/feet Small

Angiogram

Kawasaki Dz (Mucocutaneous Lymph Node Syndrome) Microscopic Polyangiitis (polyarteritis) Wegener’s Granulomatosis

ECG

ASA IVIg Ø corticosteroids

P-ANCA+

Medium, small (esp lung)

C-ANCA+

Immunosuppressive tx

Thromboangitis Obliterans (Buerger’s Dz) Raynaud’s Phenomenon 2° Raynaud’s

Medium and small aa. in extremities (radial, tibial) Medium (esp digits, nose, ears) Medium (acral)

Stop smoking IV iloprost (PG analogue) Avoid cold Ca+2 channel blockers Avoid cold Ca+2 channel blockers

Vasospasm d/t dz (SLE, Buerger’s), Systemic sclerosis, CREST,

Vasculitis
Disorder
Veins Varicose Veins

Vessels
Superficial Veins

Pathology
Abnormally dilated, tortuous vv. d/t ↑ pressure – varibly thinned and scarred (thickened) – phlebosclerosis (spotty calcification) Portal HTN, chirrosis Portal HTN, pregnancy Virchow’s Triad: stasis, endothelial injury, hypercoagulability Infection (Staph Aureus) often d/t IV insertion/infiltration Extrinsic compression of SVC (lung apex small cell ca) Incomplete basement membranes predispose to infection/tumor invasion Occlusion of lymph drainage d/t tumor, surgical removal of nodes, radiation fibrosis, filariasis, post-inflamm scarring

Presentation
>50yo, obesity, prolonged standing, post-pregnancy woman

Complications
Venous stasis, edema, pain, thrombosis, poor wound healing (ulcers), stasis dermatitis Massive hemorrhage Thrombosis and ulcers Pulmonary emboli

Other
Familial tendency

Esophageal Varices Hemorrhoids Venous Thrombosis (DVTs) Thrombophlebitis Superior Vena Cava Syndrome Lymphangitis Lymphedema

Esophageal vv. Deep leg vv, portal v., dural sinuses Superficial vv. SVC Lymph vessels Lymph vessels

Varicose vv. in esophagus Vericose veins in AJ (anal junction) Pain in leg or flexing foot, edema

Often fatal

Swelling, blue discoloration of face Red streak along lymphatic course Swelling distally GAS most common

Vascular Tumors Benign Hemangiomas Capillary Skin, oral mucosa Cavernous Pyogenic Glomus Cavernous Lymphangioma Bacillary Angiomatosis Deep structures Skin of oral mucosa Small vessels of fingertips Large, neck, axilla Skin, bone, brain, other organs

Non-encapsulated close packed capillaries – blood-filled – little stroma – may be thrombosed Large blood-filled spaces, sharply defined not encapsulated, scant stroma, can be thrombosed or calcifications Polypoid, rapidly growing, on stalk – post trauma – granulomatous histologicallyinflammation – exuberant healing process Modified smooth m. cells of glomus body – nests of glomus cells around vessels No blood in vascular lumens – On neck in Turner’s Syndrome (XO) – Non-encapsulated Opportunistic infection (Bartonella family bacteria) – proliferation of capillaries w/ endothelial cells

“Strawberry” on juvenile, flat to raised Grossly red-blue, soft and spongy, 1-2cm Von Hippel-Lindau (in brain, eye, pancreas, liver) “Lobular capillary” Painful Under fingernail Cystic Hygroma Red papules, neutrophils and bacteria, silver stain of bacilli Cat-Scratch fever

Vasculitis
Disorder Vessels Pathology
Patch: dilated irregular vessels, inflamm like granulomatous tissue Plaque: Dilated jagged vascular channels w/ plump spindle cells w/ chronic inflamm cells Nodule: same but with mitoses, hemosiderin and hemorrhage Herpesvirus 8 (KSHV) – necessary and sufficient – sexually transmitted? Lymphadenopathy, aggressive Lymph nodes, mucosa, viscera All over Endothelial

Presentation
Grossly patch→plaque→ nodules pink to purple

Complications

Other
Local excision w/ good results – more lesions= radiation, chemo, antivirals

Intermediate and Malignant Kaposi Sarcoma Skin

Chronic Lymphadenopathic Transplant AIDS Angiosarcoma

Older men, Red-purple plaques Young Africans

Ashkenazi Jews HIV+ and HIVAggressive Usually fatal Most common AIDS cancer

Death d/t infection, not KS Environmental – PVC, Arsenic, Thorotrast – Radiation induced – chronic lymphedema – local invasions and metastases Older adults, asymptomatic red nodules anywhere on body (advanced = lg. gray white fleshy masses w/ central necrosis and hemorrhage)

Mesenchymal tumor Poor survival

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

Sign up to vote on this title
UsefulNot useful