VASCULITISEK, TÜDŐ-VESE SZINDRÓMÁK GYERMEK ÉS FELNŐTT KORBAN. Dr. Túri Sándor SZTE Gyermekklinika, Szeged

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1 VASCULITISEK, TÜDŐ-VESE SZINDRÓMÁK GYERMEK ÉS FELNŐTT KORBAN Dr. Túri Sándor SZTE Gyermekklinika, Szeged

2 Renal vascular involvement in vasculitides

3 ANCA-induced vasculitis: a possible pathogenetic path

4 Crescent formation

5 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.

6 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).

7 16855/2005 Ksz cellularis félhold Intracapillaris fibrin depositio sclerosis komprimált glomerulus fibrocellularis félhold fibrocellularis félhold k ü glomerulusokban változókorú elváltozások

8 AHF Fibrin a glomerulusokban, félholdakban. AHF 16855/2005 Ksz

9 Destruction of nasal cartilago in Wegener s granulomatosis

11 Proliferativ glomerulonephritis in Churg Strauss syndrome

12 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

16 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

17 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.

18 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.

19 Renal angiogram in polyarteritis nodosa

20 Renal arteries

21 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

23 Typical molar rash of SLE. Erythema, erosion, and atrophy are present. Note sparing of nasolabial folds.

24 Clinical features of patients with evident lupus nephritis

25 Induction treatment for lupus nephritis

26 Maintenance treatment for mild lupus nephritis

27 Causes of Goodpasture s syndrome (lung hemmorhage and rapidly progressive renal glomerulonephritis)

28 Renal biopsy in Goodpasture s disease

29 16855/2005 Ksz IgG IgG C3 keringő anti-gbm

30 Chest radiographs of a patient with lung hemmorrhage

31 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 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

32 16855/2005 Ksz Félholdképződéssel járó focalisan necrotisalo és sclerotisalo GN: az összes glomerulus beteg, A acut és idült glomerularis és tubulointerstitialis károsodások. z

33 Ksz Polyangitis nodosa Félholdképződéssel járó glomerulonephritis: az összes glomerulus beteg, acut és idült glomerularis és tubulointerstitialis károsodások.

34 Ksz fibrocellularis félhold

35 POLYANGITIS NODOSA

36 Vasculitis-asszociált tüdővérzés. panca+, tüdő-vese syndroma intraalveolaris vérzés hemosziderin tartalmú macrophagok

37 első vesebiopszia B.H Ksz ANTI-GBM NEPHRITIS

38 panca első vesebiopszia B.H Ksz IgG AHF ANTI-GBM NEPHRITIS

39 második vesebiopszia: hegesedés dominál B.H Ksz

40 Koponya MRI felvételek (T2, axiális ) GOODPASTURE SYNDROMA + ANCA POZITIV VASCULITIS

41 GOODPASTURE SYNDROMA + ANCA POZITIV VASCULITIS

42 Ksz ép glomerulus ép glomerulus ép glomerulus Egyik vesebiopszia részletben több ép glomerulus.

43 Mellkasröntgen: 1. Kiindulási állapot 2. Progresszió 3. A folyamat regressziója a kezelés hatására WEGENER GRANULOMATOSIS

46 Polyarteritis nodosa

47 Fig. 1. Panarteritis nodosa

49 Periarteritis Nodosa. A type of vasculitis which frequently involves the kidneys.

50 Indirect immunofluorescence for antineutrophil cytoplasmic antibodies (ANCA)

51 Cutaneous vasculitis

52 A, the localized erythematous rash of SLE in a nonmalar distribution. B, the rash of SLE often has a slight white scale.

53 fibrin endocapillarisan és a félhold területében 16855/2005 Ksz

55 Cutaneous vasculitis in SLE. Purpuric, ulcerative, and necrotic skin lesions of active disease.

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