Pp. 143-170 (28)
Vasculitis is inflammation in vessel walls leading to poor blood circulation
and damage to vessels. The vasculitis are devided into large vessel vasculitis (Takayasu
arteritis [TAK] and Giant cell arteritis [GCA]), medium vessel vasculitis (Polyarteritis
nodosa and Kawasaki disease), small vessel vasculitis associated with antineutrophil
cytoplasmic antibody (microscopic polyangiitis [MPA], granulomatosis with
polyangiitis [GPA], eosinophilic granulomatosis with polyangiitis [EGPA]) and others.
TAK affects patients younger than 50 years, and GCA after age 50. Clinical features
include leg claudication, headaches, postural dizziness, visual disturbances and reduced
or absent upper limb pulses.
Management of TAK and GCA include high doses of GCS. Treatment of GCA can
prevent blindness due to occlusion of optic arteries. New option is tocilizumab.
Polyarteritis nodosa inflammation involves the skin, kidneys, peripheral nerves,
muscles, and gut in patients between 40-60 years old. Management includes GCS,
CYC and AZA.
Kawasaki disease is an acute febrile mucocutaneous and lymph node disease affecting
young children. Coronary arteries are often involved. Management includes IVIG and
GPA is necrotizing granulomatous inflammation involving the upper and lower
respiratory tract, necrotizing glomerulonephritis, ocular vasculitis, pulmonary
capillaritis with hemorrhage and granulomatous and nongranulomatous extravascular
inflammation is common. c-ANCA is present in 98% of patients. Management is high
doses of GCS, CYC, rituximab and AZA.
EGPA is characterized by pulmonary and systemic small vessel vasculitis, granulomas
and hypereosinophilia. Clinical features are atopic history, asthma, allergic rhinitis,
pulmonary infiltrates, mono/polyneuropathy, mononeuritis multiplex, purpura and
eosinophilia. Management is high doses of GCS, CYC and AZA.
Antineutrophil cytoplasmic antibody, AZA, CYC, Eosinophilic
granulomatosis with polyangiitis [EGPA], GCS, Giant cell arteritis [GCA],
Granulomatosis with polyangiitis [GPA], Kawasaki disease, Large vessel vasculitis,
Medium vessel vasculitis, Microscopic polyangiitis [MPA], Polyarteritis
nodosa, Rituximab, Small vessel vasculitis, Takayasu arteritis [TAK], Vasculitis.
Head of Rheumatology and Internal Diseases Department CSK MSWiA Warsaw Poland.