Blood Vessels

Pathology

Congenital Anomalies

Arteriosclerosis

HTN

Vasculitides ( inflammations)

Aneurysms & Dissections

Veins & Lymphatics

Tumors

• Vasculitides = Inflammation of Blood Vessels• Present with Non-Specific/ systemic/Vague complaints

– Fever, Myalgia, Artharlgia, Malaise, etc.,• Pathogenic Mechanisms

– Immune – MCC• 1. Immune complex = Hypersensitivity (to Drugs), Following

Viral Infections (PAN & HBV)• 2. ANCA Positive• (Anti Neutrophil Cytoplasmic Antibody) C- ANCA (Ab

Against Proteinase -3) = Wagener's– P- ANCA ( Ab against MPO) = mPAN, Chaurg – Straus

• 3. Anti – Endothelial Cell = SLE, Kawasaki’s – Infectious –

• Less Common, Direct Trauma is the cause, can be Bacterial or fungal

• Vasculitides = Types• Based on

– Size of Vessels involved– Site of involvement– Characteristic Features

• 1. Giant Cell ( Temporal ) Arteritis– Systemic Vasculitis– Sites = Temporal ( Head ache & Facial Pain), Vertebral,

Ophthalmic ( Blindness), Aorta ( Aneurysm)– Age, Sex & Ethnicity = >50 yrs., M=F, Nordic people– Clinical = Facial Pain & Headache, Diplopia & Blindness (most

dangerous, Sudden, permanent)– Pathology / Morphology = Granulomas in vessel walls, Giant

cells, Segmental involvement– Diagnosis = Biopsy is important– Treatment = Steroids save vision

1. Giant Cell ( Temporal ) Arteritis

• Systemic Vasculitis• Sites = Temporal ( Head ache & Facial Pain), Vertebral, Ophthalmic

( Blindness), Aorta ( Aneurysm)• Age, Sex & Ethnicity = >50 yrs., M=F, Nordic people• Clinical = Facial Pain & Headache, Diplopia & Blindness (most

dangerous, Sudden, permanent)• Pathology / Morphology = Granulomas in vessel walls, Giant cells,

Segmental involvement, Fragmentation of Internal Elastic Lamina (IEL)• Diagnosis = Biopsy is important• Treatment = Steroids save vision

Fragmented IEL

2. Takayasu ( Pulse less ) Arteritis

• Systemic Vasculitis = of Medium and large size vessels• Sites = Aorta ( Aneurysm), Temporal ( Head ache & Facial Pain), Vertebral,

Ophthalmic ( Blindness), • Age, Sex & Ethnicity = <40 yrs., F>M, Japanese, HLA (A24, B52, DR2)• Clinical = Pulses Weak & Low BP in Hands ( Just opposite to Coarction of

Aorta)• Pathology / Morphology = Granulomas in vessel walls, Giant cells,

Fibrosis and Lymphocytic infiltration • Diagnosis = Biopsy• Treatment = Steroids • Complications = MI, Aortic Regurgitation

3. Poly Arteritis Nodosa (PAN)

• Systemic Vasculitis = of Small & Medium size vessels• Sites = Kidneys (not the Glomerular capillaries), Heart, Liver, and GIT (NOT

LUNGS) • Age, Sex & Ethnicity = Young Adults, M>F , no special risk groups• Clinical = Ulcers, Infarcts, Hemorrhages, HBsAg Positive• Clinical course = Relapses & Remissions• Pathology / Morphology = acute (inflammation, Fibrinoid Necrosis,

Thrombosis), Chronic (modularity, Fibrosis ) • Diagnosis = Biopsy is important, No ANCA Positive• Treatment = corticosteroids and Cyclophosphamide • Complications = MCC of death – Renal Failure, CNS lesions

PAN• Small & Medium size

Vessels• Different stages of disease

in same or different vessels• HBsAg Positive• ANCA Negative• Capillaries (Pulmonary,

Glomerular) not involved,

• Large infarcts seen• Bad prognosis

4. mPAN( micro)• Smallest vessels( Arterioles,

capillaries, Venules)• Same stage of disease in all

vessels• Negative• P-ANCA Positive• Involved (Necrotizing

Glomerulonephritis, Hemoptysis)

• No Large infarcts• Better Prognosis

Microscopic polyangiitis (microscopic polyarteritis (m PAN), Hypersensitivity or Leukocytoclastic Vasculitis)

5. Kawasaki DiseaseMuco Cutaneous Lymph node syndrome

• Systemic Vasculitis = of Small & Medium size vessels• Sites = coronary, cutaneous vessels• Age, Sex & Ethnicity = Very young (<4yrs. Age), North America, Japan• Clinical = Fever, Muco (conjunctival, oral erythema, erosions), cutaneous

(erythema of palms, soles, & Skin rash), Lymph node syndrome ( cervical)• Clinical course = spontaneous Remissions in most of them • Treatment = aspirin, Immunoglobulins• Complications = coronary aneurysms

6. Churg – Strauss Syndrome

• Systemic Vasculitis = of Medium and large size vessels• Sites = Pulmonary, Coronary, Cutaneous, • Age= 40- 50 yrs. • Clinical = Allergic Rhinitis, Bronchial Asthma, Eosinophilia, skin rash • Pathology / Morphology = Eosinophilic Granulomas in vessel walls,

Necrosis, Eosinophilic infiltration of organs • Diagnosis = Biopsy• Treatment = Steroids • Complications = Myocarditis, Coronary Vasculitis

Transient Pul. Infiltrates Eosinophilic Necrosis Skin Rash

7. Wegener’s Granulomatosis

• Systemic Vasculitis = of small & Medium size vessels• Sites = Pulmonary, renal, nasal & Para nasal, • Age= 40yrs., M>F, • Clinical = Pneumonitis & Nodular Pul. Infiltrates (MC), Ch. Sinusitis,

Glomerulonephritis, Nasal ulcers• Pathology / Morphology = Granulomatous Necrotizing Vasculitis,

Crescentic Glomerulonephritis, Nasal Granulomas • Diagnosis = Biopsy, C-ANCA positive, Triad (Vasculitis, Respiratory, Renal)• Treatment = Cyclophosphamide• Complications = RPGN, Pulmonary and upper airway obstruction

8. Thromboangiitis Obliterans

( Buerger’s disease)

• Limited Vasculitis• Sites = Tibial & Radial arteries • Age= <35yrs., M>F, Smokers, Asians• Clinical = intermittent claudication, rest pain ( neural involvement),

ulcerations of Toes, Fingers • Pathology / Morphology = Granulomatous inflammation, Thrombi with

central micro abscess (Pus) • Diagnosis = Biopsy, • Treatment = Avoidance of smoking, Surgery, Prostaglandin analogues • Complications =ulcers, gangrene, infection need of amputation