The Blood Vessels –II

Inflammatory Diseases of blood vesselsTopic

Giant Cell (Temporal) Arteritis Takayasu Arteritis Wegener Granulomatosis (arteriole, capillary, venules) Polyarteritis Nodosa – (medium size artery) Thromboangiitis Obliterance- medium size artery) Hypersensitivity ( Leukocytoclastic Vasculitis): arterioles

Giant Cell (Temporal) Arteritis

Def: 1. An acute and chronic granulomatous inflammation. 2. Involvement of the artery includes

1. Temporal artery (commonest) and 2. Ophthalmic artery3. Branches of external carotid arteries.

3. Characteristically the affected artery develops nodular thickenings and may become thrombosed4. A temporal artery involved with temporal arteritis may appear prominent and tortuous as it courses

beneath the skin of the scalp. Such an artery may be palpably firm and painful.

Morphology:

5. Granulomatous inflammation of the media with lymphocytic infiltrate, giant cells( identify)

Clinical features of Giant Cell (Temporal) Arteritis

Mostly after the age of 50. Polymyalgia rheumatica, headaches, weight loss, anorexia, and tenderness. Lab: Highly increased erythrocyte sedimentation rate (ESR). Treatment: use of corticosteroid has excellent result.

Some persons will also have polymyalgia rheumatica. There are no specific laboratory findings, though the sedimentation rate is often quite elevated (as high as 100 mm/hr or more). A trial of corticosteroid therapy, if successful, may provide a presumptive diagnosis in lieu of biopsy. The inflammation is mixed with scattered giant cells. It can be quite focal, requiring serial sections even of a small biopsy. If chronic, only fibrosis may be seen.

Takayasu Arteritis

6. Def: Granulomatous vasculitis.7. Age: Females younger than 40.8. Involvement: branches of aortic arch, renal and pulmonary artery.9. Renal artery involvement may cause (secondary )hypertension

10. Morphology:11. Stenosis and or Aneurysm12. Micro: Giant cells

Clinical:13. BP and weaker pulses in the upper extremities (carotid, radial, ulnar) than in the lower.14. Ocular disturbances.15. Neurological deficits.

Wegener Granulomatosis16.17. Definition: Necrotizing Granulomatous vasculitis affecting capillaries, venules, arterioles, and arteries.

18. Morphology:Giant cells is the blood vessels in lung, kidney.

Occasional giant cells Large nodular lesions in the lung

19. Death within 1 year if untreated (malignant course).20. c-ANCA is positive in the serum in 95% & is used to monitor disease activity.

21. Clinical Features of Wagener Granulomatosis22. Nasopharynx: Mucosal ulceration, sinusitis, rupture of nasal septum.23. Kidney: Crescentic Glomerulonephritis (may lead to Hematuria and acute renal failure), hypertension. 24. Pneumonitis and hemoptysis

Polyarteritis Nodosa – (PAN)Definition:

25. Systemic vasculitis. 26. Hepatitis B surface antigens present (HBsAg).27. ANCA negative.28. Involving kidney, skin and other organs except the lungs.29.

Morphology: Fibrinoid necrosis of intima and thrombosis.

Clinical 1. Age: all age group2. Course: episodic fever, with long symptom-free intervals.3. Skin: Palpable purpura, ischemia & ulceration due to thrombosis.4. Kidney: aneurysm= bad prognosis= hypertension

Impairment of perfusion causing infarcts Small aneurismal dilations in branches Or ischemic necrosis of the renal artery

Churg-Strauss syndrome.A variant of Polyarteritis Nodosa.Features:

Involve pulmonary vasculature Eosinophilia Asthma Positive for p- ANCA

Microscopic Polyangitis

Aka: Microscopic polyarteritis, Hypersensitivity, OR Leukocytoclastic Vasculitis. Etiology: reaction to an antigen such as

drugs (e.g., penicillin), Microorganisms (e.g., streptococci) Malignancy

Leukocytoclastic vasculitis Deposition of antigen-antibody complex in vessels wall. P- ANCA positive Neutrophilic vasculitis.

o Serum Sickness

Henoch-Schönlein Purpura:

Pathology: vasculitisPurpura, arthritis and abdominal pain presentIn addition there is Mesangial IgA deposition in kidneyPresented with Hematuria and purpura.

ANCA-associated vaculitis(ANCA= Anti-Neutrophil Cytoplasmic Antibodies)

Wegener Granulomatosis (c-ANCA):o Giant cells+o No Asthma.

Churg-Strauss syndrome (p-ANCA):o Asthma & eosinophilia.o Fibrinoid necrosis

Microscopic polyangiitis (p-ANCA):o No asthma, eosinophilia or granuloma.o Neutrophil infiltration

Nice to know o Proteinase 3 (PR3): PR3 is the most common antigen target of c- ANCA o Most common p-ANCA target is myeloperocidase (MPO),

Thromboangitis Obliteransaka: Buerger Disease

Definition: Thrombus and abscess formation in the artery. Involvement: tibial and radial arteries. Age & sex: middle aged( before 35 years) adults; men > women There is a very close association with smoking.Clinical: Early: ulcer, pain on walking – claudication, Raynaud phenomenon Later: Gangrene. Treatment : stop smoking Buerger disease: the lumen is occluded by a thrombus containing two abscesses (arrow):

Treatment: = Abstinence from cigarette smoking in the early stages of the disease often prevents further attacks.

Raynaud Phenomenon

Paroxysmal Color change: white-blue-red.

Kawasaki disease

Commonly occur in young children Clinical: conjunctivitis + maculopapular skin rash + lymphadenopathy. Location: involve coronary artery and may produce coronary aneurysm. In some patient this disease may cause myocardial infarction if the coronary artery is involved. Prognosis: self limited

Systemic lupus erythematosus

• Vasculitis associated with fibrinoid necrosis.• Antiphospholipid antibody syndrome is present → may lead to venous thrombosis.• Positive ds-DNA ANA.

Venous disease: Varicose vein

Definition: dilated tortuous superficial veinsPathogenesis: increased intraluminal pressureType: superficial vein in lower limbs, esophageal varices, hemorrhoids.

Varicosities of the superficial veins in lower limbs

Abnormally dilated, tortuous veins produced by o prolonged, increased intraluminal pressure and o Loss of vessel wall support 9 like muscle paralysis).

Clinical: edema, thrombosis, ulcers. This ulcer is difficult to treat. Now-a- day’s hyperbaric therapy is conducted to heal these ulcers.

In some case inflammation of the vein can occur and we can term this as thrombophlebitis. In such occasion pain is an important clinical feature.

Other Varicosities

Esophageal varices: in patients who have cirrhosis of the liver and its attendant portal hypertension. Hemorrhoids: varicose dilation of the hemorrhoidal plexus of veins at the anorectal junction: produce

fresh blood during defecation. o Caused by repeated pregnancies or straining at stools (constipation).

Deep vein thrombosis

Cause: hypercoagulable stateso Adenocarcinoma, pregnancy, obesity, the postoperative state, immobilization, anti

phospholipid syndrome (in SLE patient) factor V Leiden etc.

Involvement: iliac, femoral, popliteal vein.o Mostly Asymptomatic( and so dengerus)o Leg swelling, erythema

Complication: saddle thrombus= acute cor pulmonale - acute onset of dyspnea and quick death.

Tumor of the blood vessels

Benign vascular tumors:

o Spider telangiectasia (arterial spider) Radial and often pulsatile array of dilated subcutaneous arteries or arterioles

with a central core. Etiology: Hyperestrinism and in liver failure

Hereditary hemorrhagic telangiectasis (Osler- Weber-Rendu disease)

Autosomal dominant. Location: present from birth and distributed widely over the skin and mucous

membranes, and GIT Clinical : spontaneous Nosebleeds, bleeding into the gut, or hematuria

Morphology: vascular malformations consisting of dilated capillaries and veins. AKA- Arterio venous Malformations (AVM) it is a vascular hamartoma. Compled of thick and thin walled blood vessels.

Capillary haemangioma

Commonest and a benign tumor Morphology: Lobulated but unencapsulated aggregates of closely packed, thin-walled capillaries. Site: skin, rarely visceral organ Clinical: red skin nodule ((strawberry like!) Prognosis: Regress spontaneously

Cavernous Haemangioma

Morphology: Made up of large, cavernous vascular spaces and dystrophic calcification. Site: Visceral organs ( e.g.: liver). Associated with von Hippel-Lindau disease/ syndrome ( chromosome three)

Von Hippel-Lindau disease: cavernous haemangioma / hemangioblastoma in cerebellum, and eye, bilateral hereditary renal cell carcinoma, cyst of liver.

Cavernous lymphangioma (cystic lymphangioma/ cystic hygroma)

Age: In children, Site: Neck or axillae Size: Up to 15 cm in diameter Morphology: made up of massively dilated, cystic lymphatic spaces. May be cause difficulty during delivery. Cystic hygroma: deformity

Angiosarcoma

Age: often in older adults, a malignant tumor Sites: Most commonly in the skin, soft tissue, breast, and liver. Marker: CD31, CD34, or vWF, FVIII, Vimentin. Morphology: proliferating vascular channels and spindle cells. Carcinogen: Arsenic, polyvinyl chloride (plastic factory worker), Thorotrast.

Kaposi's sarcoma

It is an angiosarcoma

Agent: HIV and KSHV – 8 (incorporated within the host tumor cells genome) Pathogenesis:

o Kaposi Sarcoma Herpes Virus proteins Incorporate in host genome and 1. may disrupt the control of cellular proliferation ( cell cycle) and2. Prevent apoptosis of endothelial cells, through the production of p53 inhibitors

Morphology: sheets of plump, proliferating hyper chromatic spindle cells Clinical : Multiple red to purple skin plaques or nodules, Histology of nodular form, demonstrate sheets of plump, proliferating spindle cells and extravasation

of RBC.