systemic vasculitis the presentation.pptx

systemic vasculitis

1. Systemic vasculitis (vasculitides) By Dr Nwachukwu shalom

2. definition Vasculitides are a heterogeneous group of rare autoimmune diseases characterized by blood vessel inflammation (vasculitis). Inflammation can lead to ischemia, necrosis, and/or hemorrhage, with subsequent end-organ damage. They are either primary (idiopathic) or secondary to an underlying disease (e.g., HBV infection, cancer, systemic lupus erythematosus) or drug use. They are further classified based on the size of the affected vessels: small-, medium-, or large-vessel vasculitis, or variable vessel vasculitis.

3. Etiology -Primary (idiopathic) -Secondary to another disease or drug use, e.g.: Infectious diseases Viral infection: e.g., HBV, HCV, HIV Infectious endocarditis Tuberculosis Syphilis -Drugs: e.g., hydralazine, cocaine -Malignancy: e.g., multiple myeloma, lymphoproliferative disorders -Autoimmune diseases: e.g., systemic lupus erythematosus (SLE), Sjogren syndrome, sarcoidosis

4. Large-vessel vasculitis Affects: aorta and great vessels

5. Giant cell arteritis (Temporal arteritis, Horton disease) Giant cell arteritis (GCA) is a type of autoimmune vasculitis that causes chronic inflammation of large and medium-sized arteries, in particular the carotid arteries, its major branches, and the aorta. GCA is most common in women over the age of 50 and of northern European descent, and approximately 50% of patients also have polymyalgia rheumatica.

6. Presentation Fever weight loss night sweats Fatigue Malaise new-onset headache tender, hardened temporal artery jaw claudication amaurosis fugax.

7. Management If there is strong clinical suspicion for GCA, glucocorticoids should be administered immediately, even prior to diagnostic workup if needed, to reduce the risk of permanent vision loss and cerebral ischemia Laboratory studies: ↑ ESR, specifically ≥ 50 mm/h (due to Rouleaux formation of RBCs), ↑ CRP Duplex Ultrasound Temporal artery biopsy

8. Takayasu arteritis Takayasu arteritis (aortic arch syndrome) is a systemic vasculitis of large vessels characterized by granulomatous inflammation of the aorta and its major branches. It most commonly affects Asian women < 40 years of age. Patients typically present with constitutional symptoms, decreased bilateral brachial and radial pulses, angina, and syncope. Epidemiology : 15–45 y.o, ♀ > ♂ (9:1)

9. Management MR angiography /CT angiography High-dose glucocorticoids, Methotrexate/Azathioprine/Cyclophosphamide/TNF inhibitor (infliximab)

10. Medium -vessel vasculitis Affects: arteries with muscular walls

11. Kawasaki disease Kawasaki disease is an acute, necrotizing vasculitis of unknown etiology. The condition primarily affects children under the age of five and is more common among those of Asian descent. coronary artery aneurysms are the most concerning possible manifestation as they can lead to myocardial infarction or arrhythmias.

12. Clinical Features fever for at least 5 days and one of the following ≥ 4 other specific symptoms < 4 specific symptoms and involvement of the coronary arteries Specific symptoms include: Erythema and edema of hands and feet, including the palms and soles (the first week) Possible desquamation of fingertips and toes after 2–3 weeks Polymorphous rash, originating on the trunk Painless bilateral “injected” conjunctivitis without exudate Oropharyngeal mucositis Erythema and swelling of the tongue (strawberry tongue) Cracked and red lips Cervical lymphadenopathy (mostly unilateral)

13. Treatment IV immunoglobulin (IVIG) High-dose oral aspirin IV glucocorticoids

14. Polyarteritis nodosa (PAN) Polyarteritis nodosa (PAN) is a systemic vasculitis of medium-sized vessels that most commonly affects the skin, peripheral nerves, muscles, joints, gastrointestinal tract, and kidneys, but usually spares the lungs. PAN is associated with certain viral infections, most commonly hepatitis B virus (HBV) infection.

15. Clinical features Nonspecific symptoms -Fever, weight loss, malaise -Muscle and joint pain Organ involvement -Renal involvement (∼ 60%): hypertension, renal impairment. -Coronary artery involvement (∼ 35%); ↑ risk of myocardial infarction -Skin involvement (∼ 40%): rash, ulcerations, nodules -Neurological involvement: polyneuropathy (mononeuritis multiplex), stroke -GI involvement: abdominal pain, melena, nausea, vomiting -Usually spares the lungs

16. Treatment Immunosuppressants (e.g., glucocorticoids PLUS cyclophosphamide) are indicated for all patients to achieve remission. Antiviral therapy is indicated for HBV-associated PAN.

17. Small -vessel vasculitis Affects: capillaries, arterioles and venules

18. Types of small vessel vasculitis Cutaneous small vessel vasculitis Occurs 7-10 days after certain medications (penicillin, cephalosporins, phenytoin, allopurinol) or infections (eg, HCV, HIV). Palpable purpura, no visceral involvement. Immunoglobulin A vasculitis Most common childhood systemic vasculitis. Often follows URI. Classic triad of Henoch-Schönlein purpura Hinge pain (arthralgias) Stomach pain (abdominal pain associated with intussusception) Palpable purpura on buttocks/legs Associated with IgA nephropathy (Berger disease). Treatment: supportive care, possibly corticosteroids.

19. Granulomatosis with polyangiitis Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis. Lower respiratory tract: hemoptysis, cough, dyspnea. Renal: hematuria, red cell casts. Triad: Focal necrotizing vasculitis Necrotizing granulomas in lung and upper airway Necrotizing glomerulonephritis CXR: large nodular densities Treatment: corticosteroids in combination with rituximab or cyclophosphamide