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CARDIOVASCULAR SYSTEM & AUTOIMMUNE DISEASES

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Title: CARDIOVASCULAR SYSTEM & AUTOIMMUNE DISEASES


1
CARDIOVASCULAR SYSTEM AUTOIMMUNE DISEASES
  • Praveen Kumar Shenoy

2
SLE
  • Damage by tissue-binding autoantibodies and
    immune complexes
  • A multigenic disease
  • 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

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Cardiovascular Manifestations
  • Pericarditis is the most commonly recognized
    cardiac problem30
  • 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 septal pseudo-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 Raynauds 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 nonselective NSAIDs, 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
  • TNFa - 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)

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Takayasu Arteritis
  • The pulseless disease or occlusive
    thromboaortopathy
  • Most frequently in young women.
  • Most commonly in Japan, China, India, and
    Southeast Asia.
  • Arterial stenoses 3-4 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(lt 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-MPAlike 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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