|
Vasculitis of Large Vessels |
Giant cell (temporal) arteritis
e.g. Horton's disease
(60-75) |
granulomatous arteritis of aorta or large arterial branches with
preference of extracranial parts of a.carotis, a. temporalis.
Frequently associated with rheumatic polymyalgy |
| |
Figure 25: prominent temporal artery
Figure 26 &
Figure 27: histology of temporal arteritis
Figure 28: anitbody deposits (immunofluorescense) at internal elastic lamella
|
Takayasu's arteritis
(15-25) |
granulomatous arteritis of aorta and major branches.
pulseless disease, claudicatio |
| |
Figure 29: giant cell granulomatous arteritis, carotid artery
Figure 30: antibody deposits (immunofluorescence) at internal elastic lamella
|
| Vasculitis of Medium Size Vessels |
Panarteritis nodosa (classical)
(40-60) |
systemic necrotizing arteritis of medium size or small arteries
w/o vasculitis of arterioles, venoles, or capillaries and
w/o glomerulonephritis. |
| |
Figure 31: vasculitis in PAN
Figure 32: scarring of kidney in PAN
Figure 33 &
Figure 34: renal necrotizing arteritis and glomerulitis in PAN
Figure 35 &
Figure 36: nodular changes of arteries in heart and gastric mucosa |
Kawasaki's disease
(1-5) |
arteritis of medium size arteries frequently associated with a
mucocutaneous lymph node syndrome.
Coronary areries frequently involved, occasionally also aorta
and veins. |
Primary CNS vasculitis
(30-50) |
small & medium size muscular arteries;
severe headache, progressive dementia
multifocal CNS symptomatology |
Thromboangiitis obliterans
Buerger's disease
(20-40) |
small and medium sized arteries and veins; thrombosis
associated with cigarette smoking
(not generally accepted as entity) |
| |
Figure 37 &
Figure 38: acute and chronic vasculitis in Buerger's disease |
| Vasculitis of Small Vessels |
Wegener's disease
(30-50) |
granulomatous inflammation involving the respiratory tract,
with necrotizing vasculitis of small and medium size vessels
Including capillaries, venoles, arterioles and arteries.
Frequently with necrotizing glomerulonephritis. |
| |
Figure 39: nasal deformation in advanced Wegener's disease
Figure 40: aphthous stomatitis
Figure 41 &
Figure 42: necrotizing granulomatous inflammation
Figure 43: c-ANCA left (in Wegener's) and pANCA (non-specific)
Figure 44 &
Figure 45: pulmonary involvement in Wegener's disease
Figure 46 &
Figure 47: focal glomerulitis and late renal changes in Wegener's disease |
Lymphomatoid granulomatosis
(40-60) |
polyclonal lymphoproliferation with lymphocytic vasculitis.
May progress to T-cell non-Hodgkin's lymphoma
cough, dyspnoe., B-symptoms |
| |
Figure 48 &
Figure 49: lymphomatoid granulomatosis (angiocentric lymphoma) in lung
Figure 50: cytologic detail of angiocentric lymphoma |
Churg-Strauss syndrome
(40-60) |
eosinophilic granulomatous inflammation of the respiratory tract
with necrotizing vasculitis of small and medium size vessels.
Usually asthma and blood eosinophilia. |
| |
Figure 51 &
Figure 52: pulmonary infiltrates in Churg-Strauss' disease
Figure 53: angiocentric lymphoid infiltrates
Figure 54: focally necrotizing granulomas |
Microscopic polyangiitis
(microscopic panarteritis)
(40-65) |
necrotizing vasculitis of small vessels (capillaries, venules,
arterioles) with small or minimal gimmune depotsh in situ.
Occasionally necrotizing arteritis of small, medium size and
large arteries.
Frequently necrotizing glomerulonephritis.
Frequently pulmonary capillaritis. |
Schoenlein-Henoch's purpura
(5-20) |
vasculitis of small vessels (capillaries, venules, arterioles) with
Predominantly IgA immune deposits in situ.
Usually involved are skin, intestines, glomeruli.
Arthritis or arthralgy may accompany.
(compare also hypersensitivity vasculitis with other Ig depots) |
| |
Figure 55: Arthus-type arteritis in Schoenlein-Henoch's disease
Figure 56: immune complex glomerulitis |
Essential cryoglobulinemic vasculitis
(39-82) |
vasculitis os small vessels (capillaries, venules) with deposits
of cryoglobulins in situ and cryoglobulins in serum.
Skin and glomeruli are frequently involved.
(check for HCV infection)
|
| |
see Figures 6 & 7 |
Cutaneous leukocytoclastic angiitis
(various forms, children to adults) |
isolated leukocytoclastic angiitis of the skin w/o systemic
vasculitis or glomerulonephritis
(there are also forms with systemic involvements of lungs,
kidneys, musculoskeletal system) |
| |
Figure 57: skin ulcer in leukocytoclastic vasculitis (see also fig. 4)
Figure 58: epidermal sloughing in advanced leukocytoclastic vasculitis
Figure 59 &
Figure 60: granulocytic vasculitis |
Behcet's syndrome
(30-50) |
oral, intestinal & genital ulcers, uveitis, thrombophlebitis |
| |
Figure 61: characteristic skin lesion in Behcet's syndrome |