VASCULITIS AND VASCULOPATHY Mantosh S. Rattan @CincyKidsRad facebook.com/cincykidsrad
Disclosure No relevant financial disclosures
Outline Overview Referral pathways MR imaging Case examples
Vasculitis
Vasculitis Inflammation in the blood vessel wall Rare and diverse set of diseases Diagnosis frequently challenging and delayed
Vasculitis Typical referral pathways: Patients with systemic inflammation (early) Patients with sequelae of vessel damage (late) Hemodynamic changes End organ damage
Imaging goals Establish correct diagnosis Evaluate disease extent and activity Follow treatment response Assess for complications
Vasculitis MRI protocol Blackblood T2 FS DWI 3D T1 GRE FS pre/post T1 FS pre and post MRA 3D whole heart
Mural thickening Blackblood Choe et al. AJR 2000
Mural and perivascular edema T2 FS
Mural and perivascular enhancement T1 weighted pre and post contrast
Mural restricted diffusion DWI
Luminal caliber change MRA
2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides Arthritis & Rheumatism Volume 65, Issue 1, pages 1-11, 27 DEC 2012 DOI: 10.1002/art.37715 http://onlinelibrary.wiley.com/doi/10.1002/art.37715/full#fig1
Common Pediatric Vasculitides Large vessel Medium vessel Small vessel Takayasu arteritis Kawasaki disease Polyarteritis nodosa Henoch- Schönlein purpura (IgAV)
Takayasu Arteritis Large vessel vasculitis Typically diagnosed during adolescence F>M (3:1) 3 phases: Early prepulseless phase Vascular inflammatory phase Late occlusive phase
Takayasu Arteritis Distribution: Thoracic and abdominal aorta simultaneously Renal arteries Pulmonary and coronary arteries Vascular complications: Stenosis Occlusion Aneurysm (less common)
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
Takayasu Arteritis
Takayasu Arteritis T2 FS
Takayasu Arteritis DWI
Takayasu Arteritis T1 weighted pre and post contrast
Takayasu Arteritis MRA
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. 2016 Soliman et al. Pediatric Radiology 2015
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
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
Kawasaki Disease 3D whole heart
Kawasaki Disease Perfusion-rest Perfusion-stress
Kawasaki Disease Late gadolinium enhancement
Polyarteritis Nodosa Medium and small vessel vasculitis Classic distribution: Renal arteries Hepatic arteries Mesenteric arteries Vascular complications: Stenoses Occlusions Aneurysms
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
Polyarteritis Nodosa
Polyarteritis Nodosa T1 weighted post contrast MRA
Polyarteritis Nodosa MRA
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
Henoch-Schönlein Purpura Role of imaging is primarily to detect complications Abdominal and MSK manifestations can precede skin findings
Henoch-Schönlein Purpura T2 FS T2 FS T1 weighted post contrast
Vasculopathy
Vasculopathy Vascular abnormality due to underlying connective tissue disorder (CTD) Numerous extravascular features
Vasculopathy Typical referral pathway: Patients with known or suspected connective tissue disorder
Imaging goals Screen for features of vasculopathy Follow disease status Assess for complication(s)
Vasculopathy MR protocol Blackblood Cine aortic root 3D whole heart 3D T1 GRE FS post 2D TOF 3D TOF MRA MRA
Aortic root dilation Factors affecting management: Absolute measurement Rate of change Consistent measurement technique e.g. cusp-cusp, cuspcommissure, systole/diastole Cine aortic root
Luminal caliber MRA
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
Extravascular findings Pectus excavatum Cysts Dural ectasia Chiari I
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
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)
Loeys-Dietz Syndrome TOF 3D whole heart Cine aortic root
Loeys-Dietz Syndrome TOF Blackblood Cine aortic root
Loeys-Dietz Syndrome
Ehlers-Danlos Syndrome CTD of collagen Classic clinical features: Hypermobility Skin extensibility Tissue fragility
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
Vascular EDS MRA TOF MRA
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
Marfan Syndrome TOF SSFP SSFP
Marfan Syndrome
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
Summary Vasculopathy Connective tissue diseases MR as a whole body screening exam Head/neck arterial tortuosity Aortic root dilation Extravascular findings
THANK YOU @CincyKidsRad facebook.com/cincykidsrad