Vasculitis Definition and Classification

Vasculitis

1. Dr. Fatimah Jaber Ghazwani (R2) Supervisor Dr. Ahmed Al-shahrani Vasculitis

2. Definition 04 06 03 Treatment Laboratory Testing Classification 02 01 05 Contents General Diagnostic Approach Indications for Referral

3. Definition Vasculitis refers to a heterogenous group of disorders in which there is inflammation and damage in blood vessel walls , leading to tissue necrosis.

4. Classification

5. Churg-Strauss syndrome - eosinophilic granulomatosis with polyangiitis (EGPA) Cutaneous ulceration on the elbow  B ilateral peripheral consolidation in the upper and lower lung zones High-resolution CT shows patchy bilateral ground-glass pulmonary opacities and minimal consolidation

6. Immunoglobulin A (IgA) vasculitis ( Henoch- Schönlein purpura ) Multiple purpuric papules

7. Granulomatosis with polyangiitis ( Wegener's ) subglottic stenosis related to granulomatosis with polyangiitis Chest X-ray & CT show bilateral nodules and masses , most extensive at the bases.

8. Kawasaki disease Cracked, red lips Strawberry tongue Conjunctivitis  Skin peeling usually begins under the nails

9. polyarteritis nodosa livedo reticularis Ulcerated, erythematous nodules

10. Giant cell arteritis Pallid disc swelling in GCA. raised, tender blood vessel located in the temple.

11. Takayasu aortitis Angiogram showing massive bilateral carotid dilation, stenosis , and poststenotic dilation. The MRI shows narrowing of the region of the isthmus (arrowhead) as well as diffuse irregular narrowing of the abdominal aorta (arrow).

12. The primary systemic vasculitis are difficult to diagnose because the clinical manifestations mimic several infectious, neoplastic, and autoimmune conditions . Clinical features in general can be categorized into: - Systemic signs/symptoms caused by inflammation - Visceral signs/symptoms specific to affected organ Systemic manifestations Fever Weight loss Weakness, general malaise Arthralgia, muscle pain Local/Visceral manifestations simultaneous or sequential appearance of symptoms from different affected organs. General Diagnostic Approach

13. Visceral signs/symptoms of large- and medium-vessel vasculitis In large vessel vasculitis , possibility of involvement of any size vessel should be kept in mind, because a decrease in aortic blood pressure can compromise blood flow to all downstream arteries

14. Visceral signs/symptoms of small vessel vasculitis

15. Laboratory Testing Laboratory testing is important to determine the organs involved, to exclude other diseases, and to monitor the disease and treatment toxicity.

16. Laboratory Testing CBC Patients with active vasculitis often have leukocytosis, anemia , and thrombocytopenia . Eosinophilia is a prominent feature in Churg-Strauss syndrome. look for the bone marrow suppression that may result from vasculitis treatment. Acute Phase Reactants increased ESR and an elevated CRP level are common, but are nonspecific . In patients with giant cell arteritis , an increased ESR can suggest the diagnosis and can be useful for disease monitoring. Renal Function Tests And Urinalysis Blood urea nitrogen and serum creatinine and urinalysis should be performed. Proteinuria and hematuria suggest of glomerulonephritis . Monitoring creatinine and the urinalysis is useful to detect changes in disease activity. It may be helpful to identify bladder toxicity in patients treated with cyclophosphamide .

17. Laboratory Testing Liver Function Tests Serum bilirubin and liver enzyme can provide clues for vasculitis that affects the liver, such as polyarteritis nodosa . Serial liver function tests are important in monitoring patients treated with hepatotoxic drugs , such as methotrexate and azathioprine Antineutrophil Cytoplasmic Antibodies (ANCA) C ytoplasmic ANCA is typically found in Wegener granulomatosis P erinuclear ANCA is typically found in microscopic polyangiitis and Churg Strauss syndrome . useful in monitoring response to treatment. ANCAs have been reported in other conditions (infections, IBD, and drug-induced vasculitis)

18. Imaging Chest Radiography Nonspecific abnormalities can be seen as infiltrates, nodules, patchy consolidation, pleural effusion , and cardiomegaly. Echocardiography Transthoracic echocardiography detects coronary artery abnormality in Kawasaki disease. Approximately 40% of children with Kawasaki disease have coronary artery lesions Angiography Angiography can show vascular occlusion and aneurysm. A diagnosis of polyarteritis nodosa can be confirmed by detection of aneurysms in mesenteric and renal arteries. Computed Tomography CT is of diagnostic value in patients with sinonasal Wegener granulomatosis . Nodules or masses can be seen on chest CT in approximately 90% of patients with Wegener granulomatosis.

19. Other Testing Nerve Conduction Testing Motor and sensory neuropathy can occur in the context of systemic vasculitis. Nerve conduction testing should be used for the evaluation of neurologic manifestations. Tissue Biopsy The definitive diagnosis of vasculitis is established by biopsy of the involved tissue (e.g., skin, sinuses, lung, artery, nerve, kidney. Biopsies are particularly valuable in ruling out other causes, but a negative biopsy does not rule out vasculitis.

20. Treatment

21. In primary health care, referral to specialists is needed when vasculitis presents with : Multisystem involvement (e.g., joints, skin, kidneys, nerves) Suspected large-vessel vasculitis (e.g., Giant Cell Arteritis, Takayasu arteritis) Persistent or worsening inflammation despite initial management Unclear diagnosis requiring specialized tests (e.g., ANCA, biopsy) Widespread skin ulcerations or necrotic lesions Hematuria and/or proteinuria suggestive of glomerulonephritis Scleritis or uveitis Lung nodules or infiltrates on imaging Indications for Referral to Other Specialties in Vasculitis

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