Documenti di Didattica
Documenti di Professioni
Documenti di Cultura
Pathology
Congenital Anomalies
Arteriosclerosis
HTN
Vasculitides ( inflammations)
Tumors
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
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
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
Systemic Vasculitis = of Medium and large size vessels Sites = Aorta ( Aneurysm), Temporal ( Head ache & Facial Pain), Vertebral, Age, Sex & Ethnicity = <40 yrs., F>M, Japanese, HLA (A24, B52, DR2) Clinical = Pulses Weak & Low BP in Hands ( Just opposite to Coarction of Pathology / Morphology = Granulomas in vessel walls, Giant cells, Fibrosis
and Lymphocytic infiltration Diagnosis = Biopsy Treatment = Steroids Complications = MI, Aortic Regurgitation Aorta) Ophthalmic ( Blindness),
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,
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
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
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. Wegeners 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
( Buergers disease)
Limited Vasculitis Sites = Tibial & Radial arteries Age= <35yrs., M>F, Smokers, Asians Clinical = intermittent claudication, rest pain ( neural involvement),
ulcerations of Toes, Fingers