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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, – – – – – 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 • • • • • 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 Disease Muco 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 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 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