11. VASCULITIDES-1 Year 4 Internal Medicine Notes OmpathStudy

11. VASCULITIDES-1 Year 4 · Internal Medicine Collection: 4TH YEAR ALL NOTES / IMED / IMED 4.3 / NGUGI -- Slide 1 VASCULITIS Slide 2 OUTLINE Introduction and Definition Classification Clinical manifestations Diagnosis Management Complications Questions Summary Slide 3 Introduction1 Vasculitis refers to inflammation of blood vessels. This may lead to narrowing (stenosis) and dilation (aneurysms) of the blood vessels. IgA vasculitis (Henoch Shonlein Purpura) and Kawasaki disease are the most common. HSP incidence 10-20/100,000. Slide 4 Introduction 2: “Could this be Vasculitis?” Systemic Fever, weight loss, fatigue Vascular Hypertension Absent/asymmetric pulse.\nPalpable aneurysms Tissue gangrene Cutaneous Palpable purpura, painful nodules Livedo reticularis, ulceration Slide 5 Introduction 3: “Could this be Vasculitis?” Pulmonary infiltrates or hemorrhage Neurologic Headache, mononeuritis multiplex, focal CNS signs Musculoskeletal Arthralgia, arthritis, myalgia, myositis Renal Hematuria, hypertension, renal failure Laboratory Abnormal urinalysis, ANCA, Elevated ESR, CRP Slide 6 Relatively common vasculitides Slide 7 Clinical features Slide 8 GIANT CELL ARTERITIS AND POLYMYALGIA RHEUMATICA Slide 9 Giant cell arteritis Also called temporal arteritis. Occurs in people aged <50 years. Symptoms: Pain over temporal arteries Jaw claudication when eating Visual disturbances/ visual loss.\nTreatment: steroids. Slide 10 Polymyalgia rheumatic (PMR) Is closely related to giant cell arteritis. Occurs mainly in those 70 years old. Its more common in women than men. Causes pain, stiffness and inflammation especially of muscles of the pelvic and shoulder girdles. It also causes fever, tiredness, loss of appetite, weight loss and depression. Management: steroids. Slide 11 Classification: IgA Vasculitis (HSP) Purpura mainly lower limb. Abdominal pain. Hematuria or proteinuria. Arthritis or arthralgia. IgA on biopsy. Slide 12 HSP Slide 13 IgA Vasculitis Course variable & often relapsing: symptoms relapse in upto 1/3 within 4 months.\nMay cause end stage renal disease. It’s a small vessel vasculitis with type III hypersensitivity associated with IgA deposition. Slide 14 IgA Vasculitis: clinical Palpable purpura mainly lower limb/buttock Colicky abdominal pain with vomiting, hematemesis, melena stools. Arthritis or arthralgia mainly lower limbs in upto 60%. Renal involvement in 25-60% manifesting proteinuria/hematuria. Other: orchitis, pulmonary haemorrhage. Slide 15 Kawasaki disease: Second commonest paediatric vasculitis. Leading cause of acquired heart disease in developed countries. IVIG and aspirin reduces coronary artery aneurysm from 25% to 4-9%. Aetiology is unknown. Worldwide distribution with bias to Asiatic/Japanese populations. Incidence: Japan 184.6/100,000 in under fives; and 5-8/100,000 in UK. Slide 17 KD: other clinical features Coronary manifestations the most important. Coronary involvement occurs in 15-25% cases. Irritability is another important sign. Reactivation/ erythema and induration of BCG scar. Mechanism unknown. Other features: aseptic meningitis, arthritis, sterile pyuria, Macrophage activation syndrome Slide 18 Kawasaki Slide 19 KD: Differential diagnoses Systemic JIA Scarlet fever (hand foot and mouth syndrome). Measles and other viral infections. Systemic lupus erythematosus (SLE). Slide 20 ANCA Vasculitis Slide 21 Granulomatous Polyangiitis Granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels Slide 22 Granulomatous Polyangiitis Classification Criteria Nasal or oral inflammation Oral ulcers or purulent or bloody nasal discharge Abnormal Chest X-ray Nodules, fixed infiltrates or cavities Abnormal urine sediment 5 RBC/hpf or RBC casts Granulomatous inflammation Granuloma within wall of an artery or in the perivascular or extravascular area of an artery or arteriole Presence of 2 criteria has sensitivity of 88.2% and specificity of 92.0 Leavitt et al. Arthritis Rheum 33:1101, 1990. Slide 23 Granulomatous Polyangiitis Slide 24 Airways Disease in Granulomatous Polyangiitis Sinusitis 60 % Epistaxis 50 % Saddle Nose Deformity 25 % Oral Ulcers 20 % Otitis Media 30 % Subglottic Stenosis 30 % Pulmonary Hemorrhage 30 % Data from Rottem et al (J Pediatr 1993), Belotstosky et al (Ped Nephrol 2002), and Akikusa et al. (Arthritis Rheum 2007) Slide 25 KD KD Slide 26 KD Slide 27 Systems Affected in 65 Children with Wegener’s Granulomatosis ENT 94 % Pulmonary 80 % Renal 67 % Joint 58% Ocular 54 % Skin 51 % Venous Thrombosis (16%) Data from Rottem et al (J Pediatr 1993), Belotstosky et al (Ped Nephrol 2002), and Akikusa et al. (Arthritis Rheum 2007) Slide 28 Granulomatous Polyangiitis Wung and Stone 2005; Nature Clin Pract Rheum 2:192-200 Limited Disease Confined to upper airways and lungs Severe Disease Rapidly progressive glomerulonephritis Pulmonary hemorrhage Mononeuritis Multiplex Scleritis or peripheral ulcerative keratitis Slide 29 Wung