Pediatric vasculitis
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Updates on systemic vasculitis in childhood.
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Pediatric vasculitis
- 1. Imaging of systemic vasculitis in childhood Udomluck, Thorsang, Orawan R2
- 3. Systemic vasculitis • Group of diseases • Inflammation of the blood vessels • Unclear etiology • In 2006, the European League Against Rheumatism (EULAR) and the Pediatric Rheumatology European Society (PReS) proposed an updated classification of childhood vasculitis.
- 6. Takayasu arteritis • Most common granulomatous inflammation of large arteries in children • Can be life-threatening • Early acute phase : nonspecific symptoms hypertension, fever, arthralgia • Aorta and its main branches • Arterial wall inflammation concentric wall thickening fibrosis consequent stenosis • Less commonly: aneurysm
- 7. Takayasu arteritis • EULAR/PReS criteria: angiographic abnormalities + at least 1/4 1. Decreased peripheral artery pulse or claudication of the extremities 2. Discrepancy of four limbs SBP > 10 mmHg difference in any limb 3. A bruit over the aorta or its major branches 4. Hypertension related to childhood normative data criteria
- 8. The classification of Takayasu arteritis • Type I : Supra-aortic arteries • Type IIa : Only the ascending aorta or aortic arch • Type IIb : Descending aorta with or without involvement of more proximal portions of the aorta and aortic arch • Type III : Descending thoracic aorta and abdominal portion or renal arteries • Type IV : Only the abdominal aorta or renal arteries • Type V : Generalized involvement with a combination of other types
- 16. Kawasaki disease • Acute febrile vasculitic syndrome of early childhood • Predominantly between 6 months and 4 years of age • Children outside that range Lacked the classic clinical presentation Delayed diagnosis • Medium-sized vessel systemic vasculitis : celiac, mesenteric, renal, iliofemoral, proximal upper limb arteries, coronary artery • Small aneurysms or stenotic segments
- 17. The EULAR/PReS classification criteria • Fever for 5 days (mandatory) + 4 of the following findings: (1) peripheral changes in the hands and feet (erythema, edema, desquamation, rash) or perianal area (2) polymorphus exanthema (3) bilateral conjunctival injection (4) injection of oral and pharyngeal mucosa (5) Cervical lymphadenopathy
- 18. The American Heart Association Consider the diagnosis of Kawasaki disease: fever for 5 days or more and < 4 principal features disease + coronary artery disease (detected by 2-D echocardiography or coronary angiography) A child with unexplained fever for 5 days or more + any of the principal features of Kawasaki disease
- 19. Role of imaging • Delayed treatment with IV immunoglobulin beyond 10 days of onset of fever increases the risk of coronary aneurysms • Imaging does not have a pivotal role in the initial diagnosis of Kawasaki disease • Important in the assessment of its more serious and potentially fatal complications • Echocardiography = imaging technique of choice
- 20. Echocardiography • Early phases : myo- and pericarditis & periluminal echogenicity • Subacute and convalescent phases : coronary artery aneurysms & dilatation
- 21. Cardiac MRI • Combined benefits of demonstrating coronary artery lesions & myocardial perfusion, function and viability in the same time • Detect distal coronary artery aneurysms might be missed in echocardiography
- 22. MDCT angiography of the coronary arteries • Limited high radiation dose, misleading data due to coronary calcifications (complication of longstanding coronary artery aneurysms) • Prospective ECG-triggered dual-source CT coronary angiography alternative diagnostic modality
- 23. PET scans • Limited to assess inflammation in the coronary artery wall Bec. increased glucose utilization within the heart muscle
- 24. Childhood polyarteritis nodosa (c-PAN) • Nongranulomatous anti-neutrophil cytoplasmic auto-antibody (ANCA) negative vasculitis • Affects medium- or small sized muscular arteries
- 25. The EULAR/PReS classification criteria • A positive biopsy or angiographic evidence of aneurysms or occlusion (mandatory) + 2/6 of the following findings: (1) skin involvement (superficial and deep skin infarctions, tender subcutanous nodules and livedo reticularis, which is purplish reticular discoloration pattern of the skin) (2) myalgia or muscle tenderness (3) systemic hypertension (4) mono- or polyneuropathy (5) renal involvement (proteinuria, haematuria) (6) testicular pain or tenderness
- 26. Conventional angiography using DSA • Standard imaging modality for diagnosis of PAN • Aneurysms, stenoses or occlusions of a medium- or small sized artery • Color Doppler, MR angiography and CT angiography compared to DSA, they are less sensitive and might result in false-negative • MR angiography considerable stenotic lesions in small arteries might be overestimated and diagnosed as complete occlusion
- 27. • Childhood polyarteritis nodosa. Multiple aneurysms on arteriogram and autopsy specimen
- 29. ANCA (+) Granulomatosis with polyangiitis (GPA)
- 30. Granulomatosis with polyangiitis (GPA) • Wegener granulomatosis • Small-to-medium-sized vessel ANCA-associated systemic necrotizing vasculitis • Granulomatous inflammation within arterial wall, perivascular, or extravascular area • Affects nose+paranasal sinuses, respiratory tract and the kidneys • Abs are against serine proteinase-3 Ag
- 32. • EULAR/PReS classification criteria includes 3/6 1. Hematuria or proteinuria 2. Granulomatous inflammation on biopsy 3. Nasal sinus inflammation 4. Subglottic/tracheal/endobronchial stenosis 5. Abnormal chest imaging 6. Positive PR 3 ANCA or c-ANCA staining Kidney RS Granulomatosis with polyangiitis (GPA)
- 33. Granulomatosis with polyangiitis (GPA) 1. Hematuria/proteinuria 2. Granulomatous inflammation 3. Nasal sinus inflammation 4. Subglottic/tracheal/ endobronchial stenosis 1. Abnormal chest imaging 2. PR 3 ANCA or c-ANCA staining
- 34. • Variable-sized lung nodules commonly > 5 mm (mms-10 cm) • Cavitation (common in nodules > 2 cm) with either thin or thick wall • Ground glass opacities and air space – hemorrhage/debris • Perivascular fluffy or hazy multifocal opacities • Mosaic perfusion Granulomatosis with polyangiitis (GPA)
- 35. Granulomatosis with polyangiitis (GPA) 1. Hematuria/proteinuria 2. Granulomatous inflammation 3. Nasal sinus inflammation 4. Subglottic/tracheal/ endobronchial stenosis 1. Abnormal chest imaging 2. PR 3 ANCA or c-ANCA staining
- 36. • Subglottic tracheal and bronchial stenosis • Focal/diffuse • DDx prolonged intubation, TB, amyloidosis or adenoid cystic tumors • Long-standing bronchial stenosis >> recurrent chest infection and lung collapse Granulomatosis with polyangiitis (GPA)
- 37. Granulomatosis with polyangiitis (GPA) 1. Hematuria/proteinuria 2. Granulomatous inflammation 3. Nasal sinus inflammation 4. Subglottic/tracheal/ endobronchial stenosis 1. Abnormal chest imaging 2. PR 3 ANCA or c-ANCA staining
- 38. Granulomatosis with polyangiitis (GPA)
- 39. Granulomatosis with polyangiitis (GPA)
- 40. • GPA >> necrotizing GN >> RPGN • If absence of significant imaging findings >> rely on clinical+lab tests (e.g. Bx) • CT, MRI, PET scan may reveal large-/medium-sized vessel involvement • Thrombosis, aneurysms, aortic dissection and aortic rupture Granulomatosis with polyangiitis (GPA)
- 41. ANCA (+) Microscopic polyangiitis (MPA)
- 42. Microscopic polyangiitis (MPA) • ANCA-associated vasculitis • Involves lungs and kidneys • No upper airway and non-granulomatous inflammation • p-ANCA; directed against the myeloperoxidase Ag • Pulmonary capillaritis >> pulmonary hemorrhage + renal dz
- 44. ANCA (+) Eosinophilic granulomatosis with polyangiitis (EGPA)
- 45. Eosinophilic granulomatosis with polyangiitis (EGPA) • Churg-Strauss syndrome • Necrotizing granulomatous inflammation • Involves respiratory tract • predominately affects small and medium vessels • associated with asthma and eosinophilia • ANCA positivity is more frequently seen with GN
- 46. • EULAR/PreS classification: 4/6 Eosinophilic granulomatosis with polyangiitis (EGPA) 1. Asthma 2. > 10% Eo in WBC count 3. Mono-/polyneuropathy from systemic vasculitis 4. Migratory/transient pulmonary opacities 5. Paranasal sinus abnormalities 6. Extravascular Eos in a biopsy
- 47. • Transient, bilateral consolidation with symmetrically peripheral location (90% of patients) • +/- Peribronchial or patchy random distribution • +/- Septal lines (50% of patients) Eosinophilic granulomatosis with polyangiitis (EGPA)
- 48. Eosinophilic granulomatosis with polyangiitis (EGPA)
- 49. Eosinophilic granulomatosis with polyangiitis (EGPA) http://pubs.rsna.org/doi/10.1148/radiol.10090105?url_ver=Z39.88- 2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dpubmed
- 50. Eosinophilic granulomatosis with polyangiitis (EGPA) http://www.cellmoloto.net/index.php/acmo/article/view/27181
- 51. ANCA (-) Immune complex associated small-vessel vasculitis
- 52. Immune complex associated small-vessel vasculitis • Ig A vasculitis (IgAV) • Non-granulomatous ANCA-negative vasculitis • IgA dominant immune complex deposits small vessels (capillaries, venules and arterioles) • Purpuric eruption = Henoch-Schönlein purpura • gastrointestinal tract • Arthritis • GN
- 53. Ig A vasculitis • The EULAR/ PReS classification • Presence of a palpable purpura + 1/5 1. Diffuse abdominal pain 2. Biopsy showing predominantly IgA deposition 3. Arthritis or arthralgia 4. Renal involvement (proteinuria/hematuria) 5. Negative ANCA
- 54. • Imaging findings: scanty and non-specific • Patients are usually sent for imaging because of abdominal pain • Ultrasound – small-sized mesenteric lymphadenopathy, bowel wall thickening and intraperitoneal fluid non-specific Ig A vasculitis
- 55. Ig A vasculitis
- 56. • Main role of imaging >> to evaluate possible complications • Pancreatitis • GI perforation • Bile duct necrosis • Alveolar hemorrhage • Intussusception • Secondary to Peyer’s patch hypertrophy (ileo-ileal > ileocaecal junction) • US -- a pseudomass with a target sign Ig A vasculitis
- 57. Other systemic vasculitides • Behcet disease • Secondary vasculitis • Vasculitis associated with connective tissue
- 58. Behcet disease • An immune-mediated vasculitis • Classic features: recurrent oral and genital aphthous ulcers, uveitis, skin lesions and cardiovascular manifestations • Likely patients older than 20 years of age • All sizes of both arteries and veins >> aneurysms or thrombosis
- 59. Behcet disease • Commonly seen in the pulmonary arterial tree >> Pulmonary artery aneurysms highly suggestive of Behcet disease >> Thrombosis >> Pulmonary infarctions >> Ruptured aneurysm >> severe hemorrhage • Brain • Cerebral artery -- stenotic segments >> ischemia • Thrombosis of the dural venous sinuses and venous infarcts
- 60. Behcet disease
- 61. MSK involvement in Behcet dz • Asymmetrical, nonerosive, nondeforming arthralgia/arthritis in lower extremities esp. knees • May show joint effusion and soft tissue swelling • Bone infarcts are less common
- 62. CNS findings in systemic vasculitis • Primary form -- primary CNS vasculitis • Part of a systemic inflammatory process – more common • Manifestations • Direct findings -- narrowing of the cerebral arteries • Secondary to bleeding from the diseased small vessels • MRI usually demonstrates high signal white matter foci in FLAIR and T2W • Tumor mimic lesions • Post contrast enhancement of the arterial wall = active inflammation • MRA of the cerebral vessels might fail to identify vasculitis in small vessels
- 63. CNS findings in systemic vasculitis • Normal MRI of the brain >> almost always excludes intracranial vasculitis • No pathognomonic MRI findings in vasculitis • Biopsy would be required for the diagnosis
- 64. CNS findings in systemic vasculitis: bleeding
- 65. CNS findings in systemic vasculitis
- 66. CNS findings in systemic vasculitis • DWI -- restricted diffusion in active vasculitic lesions of the brain • Acute/subacute lesions may show elevated ADC -- the lesions disappear over time suggesting a nonischemic mechanism • DDx posterior reversible encephalopathy syndrome
- 67. CNS vasculitis mimics • Non-inflammatory vasculopathies • Moyamoya disease • Post irradiation • Thromboembolic disease • Hemoglobinopathies e.g. sickle cell disease • Vasculopathies from rare genetic/metabolic d/o >> Brain biopsy is sometimes needed for a definite diagnosis
- 68. MSK findings in childhood systemic vasculitis • Arthralgia and arthritis • up to 78% of patients with IgAV • MRI helps early diagnosis of musculoskeletal changes in vasculitis • BM edema • Bone infarctions • Synovitis, arthritis, joint effusion • Insufficiency fractures
- 69. Bone infarction from large-dosed steroid use
- 70. Arthritis/synovitis from systemic vasculitis
- 71. Pulmonary findings in childhood systemic vasculitis • Wide spectrum of non-specific radiographic chest findings • Most are non-vasculitic lung diseases • Focal lesions, cavitary nodules, nodules with surrounding focal ground glass pattern (halo sign) and early peribronchial thickening • Ground glass pattern detected with CT of the lungs is more commonly seen with EGPA
- 72. Pulmonary findings in childhood systemic vasculitis • Hemoptysis + diffuse alveolar infiltrates + Hct drop • Imaging: alveolar opacities
- 73. Pulmonary vasculitis presented with peripheral ground glass densities
- 74. Differential diagnosis • Diagnosis can be challenging because of overlapping signs/symptoms and non-specific imaging findings • Unusual group of clinical manifestations involving multiple organ systems • Uveitis, rashes, arthritis or sinus troubles • RPGN • Pulmonary-renal syndrome • Arterial wall thickening • Takayasu arteritis • Noninflammatory disorders e.g. fibromuscular dysplasia • Inflammatory diseases e.g. tuberculosis, Behcet dz
- 75. • Developmental disorders such as aortic coarctation, mid-aortic syndrome and Marfan syndrome can result in aortic stenosis and aneurysms • Diffuse ground glass pattern -- nonspecific for vasculitis • DDx HP, pulmonary edema, sarcoidosis, alveolitis due to systemic sclerosis, drug toxicity, opportunistic infection Differential diagnosis
- 76. • Attenuated/occluded cerebral vessels and hyperintense FLAIR/T2W white/gray matter foci • DDx vasculitis, other inflammatory diseases e.g. viral encephalitis, Moyamoya dz Differential diagnosis
- 77. Conclusion • Systemic vasculitides are uncommon in childhood • No specific clinical features • Angiography is not of much value in evaluation of GPA, EGPA and IgAV • MRA is suggested in the assessment of Takayasu disease, Kawasaki disease and C-PAN • Chest HRCT has a role in the evaluation of GPA and EGPA • CT of the sinuses in the evaluation of GPA and CTA in the evaluation of Kawasaki disease • Color Doppler ultrasound is useful in the diagnosis and follow-up of IgAV and C-PAN
Notas do Editor
- uncommon in childhood and is frequently subjected to a delayed diagnosis Improvement in therapy and understanding of the etiopathogenesis of vasculitis have driven the need for better descriptors and groupings of diseases that have culminated in various classification criteria for vasculitis
- less commonly: aneurysm with the risk of development of mural thrombosis
- Claudication = focal muscle pain, induced by physical activity Bruit = audible murmur or palpable thrill Hypertension = > 95th percentile for height
- Type I : Supra-aortic arteries Type IIa : Only the ascending aorta or aortic arch Type IIb : Descending aorta with or without involvement of more proximal portions of the aorta and aortic arch Type III : Descending thoracic aorta and abdominal portion or renal arteries Type IV : Only the abdominal aorta or renal arteries Type V : Generalized involvement with a combination of other types
- - Evaluation of the vessel lumen is best seen by conventional angiography using digital subtraction technique (DSA), which has been considered the reference standard imaging modality - MR angiography might overestimate significant stenotic lesions showing them as occluded arteries
- Ultrasound + color Doppler is capable of evaluating the morphology of the aneurysm, which may present with turbulent flow and possible thrombosis The field of view is small in US Ultrasound cannot determine disease activity contrast to gadolinium-enhanced MRI is capable of early diagnosis of inflammatory changes in the arterial wall before significant wall thickness becomes apparent
- -
- Anti-neutrophil cytoplasmic antibody (ANCA) Granulomatosis with polyangiitis Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis
- The classic triad -- lungs: involved in 95% of cases//upper respiratory tract / sinuses: 75-90%//kidneys: 80% classical: full triad//limited: usually respiratory tract only///widespread: skin (50%)eyes (45%)peripheral nervous system (35%)heart-GI
- nose+paranasal sinuses, respiratory tract and the kidneys Chronic or recurrent sinusitis -- early manifestations Mucosal thickening of the paranasal sinuses and nasal septum perforation Erosion of the nasal turbinates and bony of the paranasal sinuses Pulmonary nodules
- PR 3 (Proteinase 3)
- nasal septum perforation and resorption of the nasal conchae (arrows) bone defects in the medial wall of the maxillary sinuses
- small cavitating pulmonary nodule with a slightly thickened wall ยังมีจุดอื่นๆอีกทั่วปอดเลย
- GPA – stridor short segment subglottic stenosis
- GPA – hemoptysis bilateral largesized pulmonary nodules with a small cavity
- GPA marked stenosis of the left bronchus (arrow) after a few years (b) shows residual left bronchus stenosis (arrow) and partial collapse of the left upper lobe
- >> pulmonary-renal syndrome
- extensive consolidation in bilateral middle and lower lung zones. CT scan shows extensive parenchymal opacities in both lungs. Poorly defined nodule (arrow) is observed in left upper lobe=hemorrhage
- bilateral symmetrical patchy confluent peripheral opacities at the upper lungs CT -- ground glass opacity at both upper lung lobes with more confluent consolidation in the left upper lung zone on a background of hyperinflated lungs
- extensive parenchymal opacities in both lungs—sparing apex CT scan shows patchy and extensive GGO in both lungs. Subpleural lungs are spared.
- patchy consolidation areas, ground glass opacities and air bronchograms.
- Ultrasound is the most common initial imaging modality used to start the investigation.
- rash and abdominal pain Gray-scale -- mild bowel wall thickening (arrow) and multiple small mesenteric lymph nodes Color Dolppler -- increased vascularity at the affected bowel segment
- No specific vessel sizes
- A 12- year-old boy who presented with diplopia, photophobia, headache and intermittent fever. Postcontrast CT scans of the brain show hypodense filling defect in the torcula herophili (arrow) consistent with dural sinus thrombosis
- usually
- direct (eg, vessel wall thickening and contrast material enhancement) indirect (eg, cerebral perfusion deficits, ischemic brain lesions, intracerebral or subarachnoid hemorrhage, and vascular stenosis)
- MRI T2 -- cerebral hemorrhagic lesions with dark signal (magnetic susceptibility artifacts) secondary to blood degradation products
- MRI (FLAIR) granulomatosis with polyangiitis (GPA) shows a cortical hyperintense lesion at the high parietal levels parasagittally 3- D MRA of the posterior cerebral circulation (b) shows attenuated beaded PCAs more accentuated on the left side (arrow) and overall attenuated distal branches suggesting a vasculitic pattern
- MRI of the brain of the Takayasu patient developed seizures MRI FLAIR -- multiple cortical and subcortical areas of increased signal intensity. No restricted diffusion MRA negative follow-up MRI FLAIR 2 weeks later หาย
- เมื่อมีอาการโดยมากจะส่งแค่ plain film เพราะ MRI ไม่เปลี่ยน management ซึ่ง plain film ก็ often negative at an early stage of the disease MRI มักส่งเมื่อ considering infection or avascular necrosis from steroid
- granulomatosis with polyangiitis (GPA) on large doses of steroids when developed ankle pain Sagittal T1 (a) and pd-fs (b) multiple bone infarcts (low signal in T1, high signal in pd-fs) with serpinginous linear margin in the distal tibial, talus and calcaneus
- granulomatosis with polyangiitis (GPA) with foot pain and swelling unenhanced sagittal T1 (a), STIR (b) and post-Gdt sagittal T1fs (c) several areas of bone marrow edema (arrow in b), enhanced nodular lesion at the dorsal navicular/medial cuneiform joint (arrows in a and c) in keeping with synovial proliferation
- An 8-year-old boy presented with hemoptysis -- peripheral ground glass densities with lack of focal nodular lesions. DDx includes vasculitis, pneumonitis and idiopathic alveolar hemorrhage. Lung biopsy showed pulmonary capillaritis.
- patient age, gender, and ethnic origin; presence of skin lesions (ulcers, palpable purpura); size of the involved vessel; involvement of other organs (especially the kidneys, lungs, and paranasal sinuses); use of medications; presence of drug abuse
- ผังช่วย DDx GPA granulomatosis with polyangiitis, MPA microscopic polyangiitis, EGPA eosinophilic granulomatosis with polyangiitis, IgAV immunoglobulin A vasculopathy, c-PAN childhood polyarteritis nodosa
- APLA = antiphospholipid antibody, HIV = human immunodeficiency virus, IgA = immunoglobulin A, PACNS = primary angiitis of the CNS, SLE = systemic lupus erythematosus.