CARDIOVASCULAR SYSTEM & AUTOIMMUNE DISEASES

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CARDIOVASCULAR SYSTEM AUTOIMMUNE DISEASES

• Praveen Kumar Shenoy

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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.

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• 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.

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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

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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.

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• 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.

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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

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• 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.

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• 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.

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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.

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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

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• 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

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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

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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

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• 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

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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.

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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

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• 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

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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.

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• 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.

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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

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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,

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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.

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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.

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• Corticosteroids are mainstay. Cyclophosphamide is
  another option.

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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.

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• 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.

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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.

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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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