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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,
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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
Fragmented IEL
• 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
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
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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
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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 Disease
Muco Cutaneous Lymph node syndrome
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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
Transient Pul. Infiltrates Eosinophilic Necrosis
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Skin Rash
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
7. Wegener’s Granulomatosis
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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)
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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
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