VASCULITISEK, TÜDŐ-VESE SZINDRÓMÁK GYERMEK ÉS FELNŐTT KORBAN Dr. Túri Sándor SZTE Gyermekklinika, Szeged
Renal vascular involvement in vasculitides
ANCA-induced vasculitis: a possible pathogenetic path
Crescent formation
SMALL VESSEL VASCULITIS 1 Wegener s granulomatosis: Microscopic polyarteritis: Churg-Strauss syndrome: Granulomatous inflammation involving the respiratory tract, and necrotizing vasculitis affecting small to medium sized vessels, Necrotizing glomerulonephritis is common. Necrotizing vasculitis with few or no immune deposits, affecting small vessels. Necrotizing arteritis involving small and medium sized arteries. Necrotizing glomerulonephritis is very common. Pulmonary capillaritis often occurs. Eosinophil rich and granulomatous inflammation involving the respiratory tract, and necrotizing vasculitis affecting small to medium sized Vessels; associated with asthma and blood eosinophilia.
RENAL MORPHOLOGICAL LESIONS IN ANCA-ASSOCIATED SYSTEMIC VASCULITIS Figure 1 (expanded view) Renal vasculitis: massive necrosis of a small artery wall with intense intraparietal and perivascular leukocyte infiltration (Trichrome X250).
16855/2005 Ksz cellularis félhold Intracapillaris fibrin depositio sclerosis komprimált glomerulus fibrocellularis félhold fibrocellularis félhold k ü glomerulusokban változókorú elváltozások
AHF Fibrin a glomerulusokban, félholdakban. AHF 16855/2005 Ksz
Destruction of nasal cartilago in Wegener s granulomatosis
Proliferativ glomerulonephritis in Churg Strauss syndrome
SMALL VESSEL VASCULITIS 2 Henoch-Schönlein purpura Vasculitis with IgA-dominant immune deposits affecting small vessels, Typically involves skin, gut and glomeruli and is associated with arthralgias or arthritis. Essential cryoglobulinemic vasculitis: Vasculitis with cryoglobulin immune deposits, affecting small vessels, associated with cryoglobulin in the serum. Skin and glomeruli are often involved. Cutaneous leukocytoclastic angitis:isolated cutaneous leukocytoclastic angitis without systemic vasculitis or glomerulonephritis
MEDIUM-SIZED VESSEL VASCULITIS Polyarteritis nodosa (classic): Kawasaki disease Necrotizing inflammation of medium- sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries or venules. Arteritis involving large, medium sized, and small arteries, and associated with mucocuteneous lymph node syndrome. Coronary arteries are often involved. Aorta and veins my be involved. Usually occurs in children
B, cross-sectional echocardiogram in the short-axis view of the aortic root in a patient with Kawasaki syndrome shows multiple large saccular aneurysms in the left anterior descending artery (LAD), as well as in the right coronary artery (RCA). A, coronary artery aneurysms.
Coronary arteritis in Kawasaki syndrome e 1B. Case #1. Higher magnification of coronary artery vasculitis. Internal elastic lamina is at the top of the frame.
Renal angiogram in polyarteritis nodosa
Renal arteries
LARGE VESSEL VASCULITIS Giant cell (temporal) arteritis Takayasu arteritis Granulomatous arteritis of the aorta and major branches, temporal artery, >50 years Granulomatous inflammation of the aorta and its major branches,<50 years
Typical molar rash of SLE. Erythema, erosion, and atrophy are present. Note sparing of nasolabial folds.
Clinical features of patients with evident lupus nephritis
Induction treatment for lupus nephritis
Maintenance treatment for mild lupus nephritis
Causes of Goodpasture s syndrome (lung hemmorhage and rapidly progressive renal glomerulonephritis)
Renal biopsy in Goodpasture s disease
16855/2005 Ksz IgG IgG C3 keringő anti-gbm
Chest radiographs of a patient with lung hemmorrhage
TREATMENT REGIMEN FOR ACUTE GOODPASTURE S DISEASE PREDNISOLON: Methyl-prednisolon pulses 1 g/m 2 i.v. CYCLOPHOSPHAMIDE: 3 mg/kg/day orally or 1 g/m2 PLASMA EXCHANGE: 80 ml/kg plasma for 5% human albumin for 14 days or until the circulating antibody is suppressed. In pulmonary hemorrhage or within 48 hours of an invasive procedure 100-300 ml fresh frozen plasma at the end of each PE MONITORING: daily blood count during PE and antibody titers remains elevated If WBC <3.5 stop cyclophosphamide Coagulation test during PE PROPHYLAXIS: oral antifungals, H-2 antagonists, cotrimoxasole (against Pneumocystis carinii Avoid non-essential lines, catheters
16855/2005 Ksz Félholdképződéssel járó focalisan necrotisalo és sclerotisalo GN: az összes glomerulus beteg, A acut és idült glomerularis és tubulointerstitialis károsodások. z
15755-19971997 Ksz Polyangitis nodosa Félholdképződéssel járó glomerulonephritis: az összes glomerulus beteg, acut és idült glomerularis és tubulointerstitialis károsodások.
15755-19971997 Ksz fibrocellularis félhold
POLYANGITIS NODOSA
Vasculitis-asszociált tüdővérzés. panca+, tüdő-vese syndroma intraalveolaris vérzés hemosziderin tartalmú macrophagok
első vesebiopszia B.H.9412-2000 Ksz ANTI-GBM NEPHRITIS
panca első vesebiopszia B.H.9412-2000 Ksz IgG AHF ANTI-GBM NEPHRITIS
második vesebiopszia: hegesedés dominál B.H. 11061-2000 Ksz
Koponya MRI felvételek (T2, axiális ) GOODPASTURE SYNDROMA + ANCA POZITIV VASCULITIS
GOODPASTURE SYNDROMA + ANCA POZITIV VASCULITIS
13772-2006 Ksz ép glomerulus ép glomerulus ép glomerulus Egyik vesebiopszia részletben több ép glomerulus.
Mellkasröntgen: 1. Kiindulási állapot 2. Progresszió 3. A folyamat regressziója a kezelés hatására WEGENER GRANULOMATOSIS
Polyarteritis nodosa
Fig. 1. Panarteritis nodosa
Periarteritis Nodosa. A type of vasculitis which frequently involves the kidneys.
Indirect immunofluorescence for antineutrophil cytoplasmic antibodies (ANCA)
Cutaneous vasculitis
A, the localized erythematous rash of SLE in a nonmalar distribution. B, the rash of SLE often has a slight white scale.
fibrin endocapillarisan és a félhold területében 16855/2005 Ksz
Cutaneous vasculitis in SLE. Purpuric, ulcerative, and necrotic skin lesions of active disease.