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Blood Vessels

Blood Vessels. 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)

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Blood Vessels

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  1. Blood Vessels

  2. 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

  3. 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

  4. 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

  5. 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

  6. 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

  7. 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

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

  9. 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

  10. 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

  11. 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

  12. 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

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