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 = 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 Systemic Vasculitis = of Medium and large size vessels Sites = Pulmonary, Coronary, Cutaneous, Age= 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. InfiltratesEosinophilic NecrosisSkin 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= 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