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10  Blood Vessels  229

             DISEASES OF THE BLOOD VESSELS


             Q. Define vasculitis.
             Ans.  Vasculitis (angiitis) is an inflammatory process involving vessels. Depending on the
             vessel involved vasculitis is labelled arteritis, capillaritis or venulitis.

             Q. Classify vasculitis.
             Ans.  Vasculitis is classified based on:
               1.  Pathogenesis
                 (a)  Direct infection
                     (i)  Bacterial,  eg,  Neisseria,  streptococci  and  staphylococci,  M.  tuberculosis  and
                       M. leprae
                    (ii)  Rickettsial, eg, Rocky Mountain spotted fever
                     (iii)  Spirochaetal, eg, syphilis
                    (iv)  Fungal, eg, aspergillosis and mucormycosis
                    (v)  Viral, eg, herpes zoster and varicella virus
                 (b)  Immunologic
                     (i)  Immune complex mediated
                       -  Infection associated, eg, hepatitis B and C viruses
                       -  Henoch–Schönlein purpura
                       -  Systemic lupus erythematosus (SLE) and rheumatoid arthritis
                       -  Drug induced
                       -  Cryoglobulinaemia
                       -  Serum sickness
                    (ii)  Antineutrophil cytoplasmic antibody (ANCA) mediated
                       -  Wegener granulomatosis
                       -  Microscopic polyangiitis (microscopic polyarteritis)
                       -  Churg–Strauss syndrome
                     (iii)  Direct antibody mediated
                       -  Good pasture syndrome (anti-GBM antibodies)
                       -  Kawasaki disease (antiendothelial antibodies)
                    (iv)  Cell mediated
                       -  Organ allograft rejection
                    (v)  Unknown
                       -  Giant cell arteritis
                       -  Polyarteritisnodosa (PAN)
                       -  Takayasu arteritis
               2.  Vessel size (2012 International Chapel Hill Consensus Conference on the Nomencla-
                ture of Vasculitis)
                 (a)  Large vessel vasculitis
                    (i)  Giant  cell  (temporal)  arteritis:  Granulomatous  arteritis  of  the  aorta  and  its
                      major branches, with predilection for extracranial branches of carotid artery
                     (ii)  Takayasu arteritis: Granulomatous inflammation of aorta and its major branches
                 (b)  Medium-sized vessel vasculitis
                    (i)  Polyarteritis  nodosa  (classic):  Necrotizing  inflammation  of  medium-sized  or
                      small arteries without glomerulonephritis or vasculitis in arterioles, capillaries
                      or venules
                     (ii)  Kawasaki disease: Arteritis involving large-, medium-sized or small arteries,
                      associated with mucocutaneous syndrome, usually seen in children
                 (c)  Small vessel vasculitis
                    (i)  Antineutrophil  cytoplasmic  antibody  (ANCA)-associated  vasculitis:  Patients
                      with circulating antibodies to neutrophilic cytoplasmic antigens.
                     (ii)  Wegener  granulomatosis  with  polyangiitis:  Granulomatous  inflammation
                      involving  the  respiratory  tract  with  necrotizing  vasculitis  affecting  small  to
                      medium-sized vessels. Necrotizing glomerulonephritis is common.




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