VASCULITIS AND VASCULOPATHY

Transcription

1 VASCULITIS AND VASCULOPATHY Mantosh S. facebook.com/cincykidsrad

2 Disclosure No relevant financial disclosures

3 Outline Overview Referral pathways MR imaging Case examples

4 Vasculitis

5 Vasculitis Inflammation in the blood vessel wall Rare and diverse set of diseases Diagnosis frequently challenging and delayed

6 Vasculitis Typical referral pathways: Patients with systemic inflammation (early) Patients with sequelae of vessel damage (late) Hemodynamic changes End organ damage

7 Imaging goals Establish correct diagnosis Evaluate disease extent and activity Follow treatment response Assess for complications

8 Vasculitis MRI protocol Blackblood T2 FS DWI 3D T1 GRE FS pre/post T1 FS pre and post MRA 3D whole heart

9 Mural thickening Blackblood Choe et al. AJR 2000

10 Mural and perivascular edema T2 FS

11 Mural and perivascular enhancement T1 weighted pre and post contrast

12 Mural restricted diffusion DWI

13 Luminal caliber change MRA

14 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides Arthritis & Rheumatism Volume 65, Issue 1, pages 1-11, 27 DEC 2012 DOI: /art

15 Common Pediatric Vasculitides Large vessel Medium vessel Small vessel Takayasu arteritis Kawasaki disease Polyarteritis nodosa Henoch- Schönlein purpura (IgAV)

16 Takayasu Arteritis Large vessel vasculitis Typically diagnosed during adolescence F>M (3:1) 3 phases: Early prepulseless phase Vascular inflammatory phase Late occlusive phase

17 Takayasu Arteritis Distribution: Thoracic and abdominal aorta simultaneously Renal arteries Pulmonary and coronary arteries Vascular complications: Stenosis Occlusion Aneurysm (less common)

18 Takayasu Arteritis EULAR/PReS Diagnostic Criteria: Angiographic abnormalities of the aorta or its main branches or pulmonary arteries + at least one of the following: Absent peripheral pulses or claudication Blood pressure discrepancy in any limb Bruits Hypertension Elevated acute phase reactants

19 Takayasu Arteritis

20 Takayasu Arteritis T2 FS

21 Takayasu Arteritis DWI

22 Takayasu Arteritis T1 weighted pre and post contrast

23 Takayasu Arteritis MRA

24 Kawasaki Disease Medium vessel vasculitis Typically 6 months to 4 years of age Cardiovascular complications Coronary artery aneurysm Pericarditis, myocarditis, valvar disease Friedman et al. JAHA Soliman et al. Pediatric Radiology 2015

25 Kawasaki Disease Coronary artery aneurysm Major cardiac events 5% of patients Size: Small Medium Giant (>8mm) Aneurysm size at diagnosis highly predictive of regression rate Morphology: Fusiform Saccular Friedman et al. JAHA. 2016

26 Kawasaki Disease ECHO Imaging technique of choice MRI Simultaneous evaluation of coronary artery lesions, myocardial perfusion, function, and viability Soliman et al. Pediatric Radiology 2015

27 Kawasaki Disease 3D whole heart

28 Kawasaki Disease Perfusion-rest Perfusion-stress

29 Kawasaki Disease Late gadolinium enhancement

30 Polyarteritis Nodosa Medium and small vessel vasculitis Classic distribution: Renal arteries Hepatic arteries Mesenteric arteries Vascular complications: Stenoses Occlusions Aneurysms

31 Polyarteritis Nodosa EULAR/PReS Diagnostic Criteria: Angiographic abnormalities or positive biopsy + at least two of the following: Skin involvement Myalgia Mono or polyneuropathy Hypertension Renal involvement (proteinuria, hematuria) Testicular pain or tenderness

32 Polyarteritis Nodosa

33 Polyarteritis Nodosa T1 weighted post contrast MRA

34 Polyarteritis Nodosa MRA

35 Henoch-Schönlein Purpura (Immunoglobulin A vasculitis) Small vessel vasculitis Most common childhood vasculitis Typically 3 to 15 years of age Skin (purpura), arthralgias, abdominal pain, renal disease Palpable purpura mandatory for diagnosis

36 Henoch-Schönlein Purpura Role of imaging is primarily to detect complications Abdominal and MSK manifestations can precede skin findings

37 Henoch-Schönlein Purpura T2 FS T2 FS T1 weighted post contrast

38 Vasculopathy

39 Vasculopathy Vascular abnormality due to underlying connective tissue disorder (CTD) Numerous extravascular features

40 Vasculopathy Typical referral pathway: Patients with known or suspected connective tissue disorder

41 Imaging goals Screen for features of vasculopathy Follow disease status Assess for complication(s)

42 Vasculopathy MR protocol Blackblood Cine aortic root 3D whole heart 3D T1 GRE FS post 2D TOF 3D TOF MRA MRA

43 Aortic root dilation Factors affecting management: Absolute measurement Rate of change Consistent measurement technique e.g. cusp-cusp, cuspcommissure, systole/diastole Cine aortic root

44 Luminal caliber MRA

45 Vascular tortuosity Vertebral tortuosity index (VTI) VTI associated with More severely dilated aortic root Increased rate of cardiac surgery Younger age at dissection, cardiac surgery, and death TOF Morris et al. Circulation 2011

46 Extravascular findings Pectus excavatum Cysts Dural ectasia Chiari I

47 Loeys-Dietz Syndrome Initially described in 2005 Connective tissue disorder (CTD) involving transforming growth factor pathway Predisposes to aortic and arterial aneurysms Classic clinical features: Hypertelorism Bifid uvula/cleft palate

48 Loeys-Dietz Syndrome Widespread arterial involvement Vascular complications: Tortuosity Aneurysm Dissection Aggressive course Lower threshold for aortic surgery than other CTD and degenerative aortic disease (4-4.5 cm)

49 Loeys-Dietz Syndrome TOF 3D whole heart Cine aortic root

50 Loeys-Dietz Syndrome TOF Blackblood Cine aortic root

51 Loeys-Dietz Syndrome

52 Ehlers-Danlos Syndrome CTD of collagen Classic clinical features: Hypermobility Skin extensibility Tissue fragility

53 EDS Vascular EDS (type 4) most commonly associated with vascular complications Medium and large arteries in any location Vascular complications: Aneurysms Dissections Ruptures Fistulae Notable extravascular manifestations: Intestinal and uterine rupture

54 Vascular EDS MRA TOF MRA

55 Marfan Syndrome CTD of fibrillin-1 Distribution: Aortic root, thoracic and abdominal aorta Pulmonary arteries Vertebral arteries Cardiovascular complications: Tortuosity Aneurysm Dissection Rupture Mitral valve prolapse Notable extravascular manifestations: Skeletal Ocular 80% or morbidity related to aortic aneurysm and dissection Aortic dimension 5 cm aortic root replacement

56 Marfan Syndrome TOF SSFP SSFP

57 Marfan Syndrome

58 Summary Vasculitis Rare in the pediatric population Most common HSP, Kawasaki, Takayasu Imaging findings play an important role Diagnosis (TA and PAN) Complications (Kawasaki) MR imaging emphasis on the vessel wall

59 Summary Vasculopathy Connective tissue diseases MR as a whole body screening exam Head/neck arterial tortuosity Aortic root dilation Extravascular findings

60 THANK facebook.com/cincykidsrad