CARDIOVASCULAR SYSTEM & AUTOIMMUNE DISEASES

1. CARDIOVASCULAR SYSTEM & AUTOIMMUNE DISEASES Praveen Kumar Shenoy

2. SLE • Damage by tissue-binding autoantibodies and immune complexes • A multigenicdisease • More common in women and can occur at any age. • Diagnosis of SLE is based on characteristic clinical features and autoantibodies. • SLE may involve one or several organ systems; over time, additional manifestations may occur

4. Cardiovascular Manifestations • Pericarditis is the most commonly recognized cardiac problem[30%] • Coronary arteritis, resulting in ischemic syndromes, rarely occur. • In SLE, myocardial infarctions are primarily manifestations of accelerated atherosclerosis. • Risk factors for accelerated atherosclerosis include disease duration, period of time treated with corticosteroids, postmenopausal status and hypercholesterolemia.

5. Additional causes of ACS in SLE include thrombosis, often related to the presence of APLA, and embolism from NBTE(Libman-Sacks). • Valvular pathology in SLE is common. • PAH can occur • Pericardial effusion , myocarditis & arrythmias are rare. • Babies born to mothers with SLE and have an increased incidence of congenital complete AV block.

6. TREATMENT • No single treatment. • Life-threatening organ involvement →high-dose corticosteroids, +/- cyclophosphamide, azathioprine, or mycophenolate. • Myocarditis with depressed left ventricular function, pericarditis with impending tamponade – an indication for cytotoxics • Surgery

7. Antiphospholipid Antibody Syndrome • Defined as the presence of either APLA or a lupus anticoagulant and a history of otherwise unexplained recurrent venous or arterial thrombosis, or frequent second or third trimester miscarriages. • Cardiac manifestations include thrombotic CAD, intracardiac thrombi, and NBE. Heart valve abnormalities occur in approximately 30 percent of patients with primary APLAS and include leaflet thickening, thrombotic masses extending from the valve ring or leaflets, or vegetations.

8. Pulmonary hypertension can occur in patients with APLA secondary to chronic thromboembolic disease. APLA may promote pulmonary artery intimal proliferation. • Catastrophic APLA • Revised Sapporo classification criteria – for diagnosis • Treatment – Anticoagulation • In catastrophic APS other options may be considered in desperate situations.

9. Systemic sclerosis • Chronic systemic disorder of unknown etiology. • Early stage → prominent inflammation, followed by widespread functional and structural alterations in multiple vascular beds and progressive visceral organ dysfunction due to fibrosis. • Mainly 2 subtypes

10. Pericardial involvement is common in PSS, and includes fibrinous pericarditis in up to 70 percent of patients at autopsy. • The presence of moderate or large pericardial effusions is an independent risk factor for mortality. • Cardiac involvement in SSc may be due to ischemic damage, myocarditis, replacement fibrosis, systemic hypertension, and PAH. • Myocardial involvement may be due to myocardial ischemia, fibrosis, and myocarditis.

11. Ventricular conduction abnormalities are common and, along with a septalpseudo-infarct pattern, correlate with reduced myocardial function with exercise. • Renal crisis may be associated with minimal or extreme hypertension, rapidly rising creatinine level, microangiopathy, thrombocytopenia, and left ventricular failure. • Pulmonary hypertension occurs in both limited scleroderma and PSS • Outcome in SSc-associated PAH is considerably worse.

12. Treatment • No therapy to alter natural history. • Immunomodulators – only cyclophosphamide of pronven role in RCT • Steroid use is ideally restricted to patients with myositis, symptomatic serositis, the early edematous phase of the skin disease, refractory arthritis, and tenosynovitis. • The lowest possible therapeutically effective dose should be used as renal crisis may be precipitated. • Various options for Raynaud’s & PAH.

13. Rheumatoid Arthritis • Most common form of chronic inflammatory polyarthritis • Chronic symmetrical polyarthritis that affects small and large joints • Affects pericardium mostly. Chronic, asymptomatic effusive pericardial disease is more common • Does not usually cause clinically significant myocarditis but CHF seen with increased prevelance

14. Secondary amyloidosis – rare, It can cause cardiomyopathy & AV block. • Leading cause of death is cardiovascular disease, with a relative risk of at least 2. • Potential risk factors for CAD in patients with RA - the chronic systemic inflammatory state, generation of proatherosclerotic HDL forms, use of selective or nonselectiveNSAIDs, under-usage of aspirin, and use of steroids, which may accelerate atherosclerosis. • Coronary arteritis & valve involvement - rarely reported

15. Treatment • Disease-modifying therapy - methotrexate, sulfasalazine, leflunomide, hydroxy-chloroquine, and low-dose prednisolone is frequently used. • Clinical pericarditis - use of NSAIDs, intensified systemic immunosuppressive therapy, pericardial steroid injections, or pericardiocentesis if hemodynamic compromise occurs. • Recurrent pericardial effusions may require a pericardial window. Constriction should be surgically treated. • Be vigilant with newer agents in presence of CHF

16. Ankylosing spondylitis • Chronic inflammatory disease of unknown cause associated HLA-B27 • TNFα- plays a central role in the immuno-pathogenesis of AS. • Features - Low back pain and stiffness, enthesitis, chest pain, joint involvement, uveitis, slowly progressive fibrosis of the upper lobes of the lungs, neurological syndromes, renal involvement & osteoporosis

17. Aortic root disease- reported in up to 100 percent of AS patients who also had aortic valve involvement in an autopsy series. Characteristic findings - thickening of the aortic root with subsequent dilation. Aortic cusp nodularity with proximal thickening seen. • Cardiac conduction disease has been well described & more common in males • Pericarditis & CAD rare • Treatment – Drugs, surgery

18. Polymyositis and Dermatomyositis • Localized or generalized myocardial dysfunction is common by echocardiographic assessment, but infrequently causes clinical failure. • The cardiomyopathy may be steroid-responsive. • PM and dermatomyositis frequently affect the conduction system.

19. Sarcoidosis • Granulomatous inflammatory disease of unknown cause. • Pericarditis though uncommon – usually clinically insignificant • Granulomatous infiltrative disease of the myocardium is often asymptomatic, but can cause arrhythmias, conduction disease and, rarely, otherwise unexplained congestive heart failure

20. Pulmonary artery hypertension and cor pulmonale can occur in sarcoidosis, generally as a result of pulmonary fibrosis. • Systemic vasculitis - an uncommon complication of sarcoidosis. • Sarcoid vasculitis can affect small- to large-caliber vessels, including the aorta. • Treatment - corticosteroids

21. VASCULITIS • Heterogeneous group of disorders linked by the primary finding of inflammation within blood vessel walls. • Can be primary or secondary • Constitutional symptoms: fever, weight loss, malaise, arthralgias/arthritis (common to vasculitides of all vessel sizes)

24. Takayasu Arteritis • The pulseless disease or occlusive thromboaortopathy • Most frequently in young women. • Most commonly in Japan, China, India, and Southeast Asia. • Arterial stenoses3-4 times more often than aneurysms. Claudication (upper [60 %]versus lower extremities [30 %]) is the most common complaint and bruits (approximately 80 percent), blood pressure, and pulse asymmetries (60-80 %) are the most common findings.

25. Aneurysms are most common and clinically most significant in the aortic root, where they can lead to valvular regurgitation (approximately 20 percent) • Hypertension is most often caused by renal artery stenosis, but can also be associated with suprarenal aortic stenosis or a chronically damaged, rigid aorta. • Cardiac, renal, and central nervous system (CNS) vascular diseases are the principal causes of severe morbidity and mortality. • Coronary artery vasculitis is rare(< 5 %), & is most frequent in the ostial regions.

26. Treatment • Corticosteroids are the cornerstone of treatment of active TA. • Initial dose of prednisone is continued for 4 to 12 weeks before commencing a gradual taper. • Frequently requires revascularization procedures • Surgical intervention should be deferred until TA is in remission • Bypass surgery yields better results than angioplasty. With bypass graft procedures, autologous vessels give better results than synthetic grafts

27. Giant Cell Arteritis • Cause of GCA remains unknown, the inflammatory lesion begins in the adventitia. • Most characteristic features of GCA are new onset of atypical and often severe headaches, scalp and temporal artery tenderness, acute visual loss, polymyalgia rheumatica, and pain in the muscles of mastication. • GCA may produce clinically apparent aortitis in ~15 % of cases and involve the primary branches of the aorta, especially the subclavian arteries,

28. Treatment • Prednisone (0.7 to 1 mg/kg/day) will reduce symptoms within 1 to 2 days and often eliminate symptoms within 1 week. About 2 to 4 weeks after clinical and laboratory para-meters, tapering of CS can begin.

29. Churg-Strauss Syndrome • Rare syndrome that includes a history of asthma, eosinophilia, pulmonary infiltrates, upper airway inflammation, and a variable frequency of renal, neurological, cutaneous, and cardiac involvement. • Cardiac disease in CSS is the most common cause of death. It is reported in 15 to 55 percent of cases and may include pericarditis, myocarditis, and coronary arteritis. Congestive heart failure occurs in 15 to 30 percent of cases.

30. Corticosteroids are mainstay. Cyclophosphamide is another option.

31. Polyarteritis Nodosa • Nongranulomatous disease of only medium-sized arteries. • Necrotizing changes seen, with weakening of the vessel wall and aneurysm formation or myointimal proliferation, causing stenosis and occlusion.

32. Features include • painful nodules (similar to erythema nodosum) or • infarction and gangrene (30 to 50 percent), • neuropathy (especially mononeuritis multiplex, 20 to 50 percent), • renal infarction and insufficiency (approximately 10 to 30 percent), • hypertension (approximately 30 percent), • segmental pulmonary infarctions (less than 40 percent), and • cardiac disease (10 to 30 percent; congestive failure, angina, infarction, pericarditis). • PAN-MPA–like spectrum obligates a search for bacterial and fungal infections as causes of endocarditis or endovascular vegetations.

33. Kawasaki Disease • Acute febrile systemic illness of childhood. • Features – fever, conjunctivitis, adenopathy, rash, mucocutaneous changes & others • Cardiac abnormalities - pericardial effusions (~30 %), myocarditis, mitral regurgitation (~ 30 %), aortitis and aortic regurgitation (infrequent), congestive heart failure, and atrial and ventricular arrhythmias. • Deaths usually result from acute coronary artery thrombosis in aneurysms that form following vasculitis.

34. Treatment • High dosages of aspirin and IVIG • Aspirin - 80 to 100 mg/kg/day until the patient is afebrile After fever subsides, the dose of aspirin is reduced (3 to 5 mg/kg/day) to achieve primarily antiplatelet effects. • Lifelong aspirin if aneurysms persist

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