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[email protected] Vasculitis Vasculitis Dr Basavaraj P Bommanahalli Assistant Professor Dept of Pathology SSIMS RC Davanagere Karnataka
| Date post: | 11-May-2015 |
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Vasculitis Vasculitis
Dr Basavaraj P Bommanahalli
Assistant Professor
Dept of Pathology
SSIMS RC Davanagere Karnataka
• Def: inflammation of wall of the vessels
• c/f constitutional signs & symptoms
fever, myalgia, arthralgia
signs of ischemia
• Pathogenesis • Direct infection:
– Bacterial– Rocky Mountain Spotted fever – Syphilis – Fungal : aspergillosis, mucormycosis – Viral: herpes zoster- varicella
• Immunologic – Immune complex mediated – Antineutrophil cytoplasmic antibody (ANCA)
mediated – Cell mediated – Direct antibody mediated
• Idiopathic
Classification
• Large vessel vasculitis • Giant cell arteritis• Takayasu arteritis
• Medium sized vessel vasculitis • Polyarteritis nodosa• Kawasaki disease
• Small vessel vasculitis • Wegener granulomatosis • Churg- stauss syndrome • Microscopic polyangitis
Giant cell (temporal) arteritis • Most common systemic arteritis • Acute, chronic granulomatous lesion
• Large arteries • Aorta & its branches • Temporal artery • Vertebral artery• Ophthalmic artery
• Segmental involvement • Nodular thickening & narrow lumen • Granulomatous inflammation
• Giant cells (foreign body & Langhans)• Lymphocytes
• Fragmentation of internal elastic lamina • Later fibrosis
C/F • Always > 50 yrs • Increased ESR• Pain & tenderness, nodular temporal A• Black outs, permanent visual loss • Biopsy is confirmatory • Anti-inflammatory drugs are effective
Takayasu arteritis • Granulomatous inflammation • Characterised by
– Ocular disturbances – Weakening of pulses in the upper extremities
pulseless disease – Fibrous thickening of arch of aorta & branches
• Associated with HLA A24-B52-DR2
• Arch of aorta & its branches • Pulmonary artery • Coronary & renal arteries
• Morphology similar to giant cell arteritis with necrosis
• Younger age group
C/F • Lower BP & weak pulses in upper limbs • Total blindness • HTN • Claudication
Polyarteritis nodosa (PAN)
• Small & medium sized muscular arteries • Most commonly visceral & renal arteries
involved • Arterioles, capillaries & venules are not involved • Segmental transmural necrotising inflammation
– Neutrophils– Eosinophils– Mononuclear cells – Fibrinoid necrosis
C/F • Fever• Myalgia • Weight loss • HTN• 30% are associated with hepatitis B • Renal involvement is seen • No glomerulonephritis
Kawasaki disease
• Young children (<4yrs) & infants • Coronary arteries are involved • Associated with mucocutaneous lymph node
syndrome – Fever– Conjunctival & oral erythema– Skin rashes – Cervical lymphadenopathy
• PAN like inflammation
• Coronary vasculitis• Coronary aneurysms • Rupture / thrombosis • MI, sudden death
Wegener granulomatosis • Classic type
Triad – Acute necrotising granulomas of upper respiratory
tract– Granulomatous vasculitis of lung & upper respiratory
tract – Focal necrotising crescentic glomerulonephritis
• Limited type: restricted to respiratory tract
• Pathogenesis – Immune complex mediated – Cell mediated
• C/F – M >F– 4th – 5th decade– c-ANCA + in 95% of cases
Thromboangitis obliterans / Buerger disease
• Characterised by – Segmental thrombosing acute & chronic
inflammation of medium sized & small arteries : tibial/ radial a
– Extending to veins & nerves of extremities
• Begins < 35yrs• Strongly associated with smoking
– Hypersensitivity to intradermally tobacco extract
• Associated with HLA-A9, HLA-B5 • Prevalent in Israel, Japan, India than US &
Europe
• Segmental acute & chronic vasculitis • Lower & upper extremities • Thrombosis – recanalisation • Contiguous involvement of veins & nerves
C/F • Gangrene • Chronic ulcerations • Severe pain: resting pain : nerve involvement
Raynaud phenomenon :• Paroxysmal pallor, cyanosis of digits of hands or
feet and tips of nose & ear • Due to cold induced vasoconstriction
precapillary arterioles & digital arteries
• Long standing cases associated with atrophy of skin, subcutaneous tissue & muscle
Primary
• Exaggeration of normal central and local vasomotor response to cold & emotion
• 3-5% of population• Median age-14yrs• No arterial pathology
Secondary
• Associated with SLE, systemic sclerosis, atherosclerosis, Buerger disease
• > 30 yrs • + vascular pathology
Tumors of vessels Benign neoplasms/ developmental /acquired conditions • Hemangioma
– Capillary– Cavernous – Pyogenic
• Lymphangioma• Vascular ectasia• Bacillary angiomatosis
Intermediate grade neoplasms• Kaposi sarcoma • Hemangioendothelioma
Malignant neoplasm• Angiosarcoma • Hemangiopericytoma
Kaposi sarcoma • Low grade malignancy• Locally aggressive • Variants
– Classic/ European type– Lymphadenopathic / African/ endemic – Immunosuppression associated – AIDS associated type
Classic/ European type• Eastern Europe • Not associated with HIV• But homosexuals are at risk
• Localised to skin
Lymphadenopathic / African/ endemic • Bantu families • Localised / generalised lymphadenopathy• Sparse skin lesions • Seen in both HIV +/ -
• Extremely aggressive
Immunosuppression associated• Transplant associated • LN + mucosa + visceral organs
• Skin lesions are absent
AIDS associated • Seen in 1/3 rd of AIDS pts • Male homosexuals • LN + mucosa + skin + viscera • Early widespread dissemination
Gross • Patch • Plaque • Nodular lesions
Microscopy • Dilated irregular, angulated blood vessels lined
by endothelial cells • Spindled cells containing pink hyaline globules• Hemosiderin laden macrophages • Mitotic figures
• KS associated Herpes virus (KSHV) / HHV-8
– Inhibits apoptosis – Increases production of p53 inhibitors
• Rx : excision + radiotherapy + chemotherapy
