Blood Vessels

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Blood Vessels
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(No Transcript)
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• 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, Kawasakis
• Infectious
• Less Common, Direct Trauma is the cause, can be
  Bacterial or fungal

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• 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 gt50 yrs., MF, 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

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1. Giant Cell ( Temporal ) Arteritis
Fragmented IEL

• Systemic Vasculitis
• Sites Temporal ( Head ache Facial Pain),
  Vertebral, Ophthalmic ( Blindness), Aorta (
  Aneurysm)
• Age, Sex Ethnicity gt50 yrs., MF, 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

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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 lt40 yrs., FgtM, 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

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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, MgtF , 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

• 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

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Microscopic polyangiitis (microscopic
polyarteritis (m PAN), Hypersensitivity or
Leukocytoclastic Vasculitis)
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5. Kawasaki DiseaseMuco Cutaneous Lymph node
syndrome

• Systemic Vasculitis of Small Medium size
  vessels
• Sites coronary, cutaneous vessels
• Age, Sex Ethnicity Very young (lt4yrs. 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

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6. Churg Strauss Syndrome
Transient Pul. Infiltrates
Eosinophilic Necrosis
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

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7. Wegeners Granulomatosis

• Systemic Vasculitis of small Medium size
  vessels
• Sites Pulmonary, renal, nasal Para nasal,
• Age 40yrs., MgtF,
  
• 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

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8. Thromboangiitis Obliterans ( Buergers
disease)

• Limited Vasculitis
• Sites Tibial Radial arteries
• Age lt35yrs., MgtF, 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