Case presentation Rheumatology Unit Selayang Hospital

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Case presentationRheumatology UnitSelayang
Hospital
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• HBR
• 18 /M/M DOA4/11/04
• DOD
  10/12/04
• feeling unwell tired x 9/12
• fever on and off 2-3/12
• no chill/ rigors
• c/o diarrhoea - 2/12
• - contain mucus - yellowish
• - no blood/malena
• LOW/LOA - 2/12 10kg
• otherwise no dysuria/frequency/ flu/cough
• denied oral ulcer/malar rash/jt pain but noticed
  rashes over the shin x 2/12

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• denied high risk behaviour
• Grand father had TB x 5yrs ago
• Denied any other forms of contact of TB
• PMH-has been seen in HKL 3/12 earlier for the
  above problem, first hosp. admission
• SH- non smoker, non alcoholic, studying in a
  college
• FH- 2nd 4 siblings, rest are well, no
  consanguineous marriage
• DH-nil

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• P/E
• Young gentleman, oriented to time/place/person
• T - 39.3
• BP - 110/60 PR -120 RR 22min
• pale, no clubbing
• no neck stiffness
• No thyroid swelling
• Rt submandibular LN - small, non tender
• vasculitic lesion on both UL and LL
• mouth - ulcer at high palate
• fundus- normal

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• CVS - gallop rhythm
• lung- clear
• PA- dull traube space
• LL
• skin lesion LL bilat - multiple
  hyperpigmented crusted lesion with central ulcer
• no pitting oedema
• Peripheral pulse

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cranial nerves intact

• UL
• Lt wrist flexion/extension 3
• Lt bicep flexion 4
• Lt shoulder abduction 3
• Rt upper limb 5/5

• LL
• Rt LL- 5/5
• Lt hip flexion 2
• Lt knee extensor 3

Reflexes hyperreflexic at all
Lt clonus for gt 3 beats on Lt leg
Rt side no clonus Sensation intact
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• Results.............
• FBC 14.49 (92) 5.7 17.2 77.1 123 0.3
• RP 17.6 123 5.0 156
• LFT 81 31 10 70 19
• Cardiac profile 1,399 383 70
• CRP 6.36 ESR 37

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• Gram positive cocci sepsis
• UR/FEME prot RBC dysmorphic RBC
• ECG sinus tachycardia

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• CXR- normal
• Ctscan - There is a hypodense lesion seen in the
  right parietal lobe.
• No intracranial haemorrhage.
• The ventricles, sulci and basal cisterns are
  normal. No midline shift.
• Right parietal lobe infarct.

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• Lumbar puncture
• OP -13mm H2O
• Appearance clear
• Glu 1.4(2.5-5.0)
• Prot 1.76(0.15-0.4)
• Chl 129(120-130)
• AFB smear -negative
• Indian Ink- negative

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Treatment..

• IV Ceftriaxone 2 g bd
• IV acyclovir 500 mg tds
• IV cloxa 2 g qid
• vitC/ferrous sulpahate/folate
• T.aspirin 75 mg OD

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MRI

• tuberculous meningitis - as evidenced by marked
  enhancement of meninges with tuberculoma/abscess.
  
• DD Fungal meningitis
• Some of the focal lesions could represent
  ischemic areas secondary to vasculitis which are
  usually subcortical in location

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• Lumbar puncture
• OP -13mm H2O - rpt 24
• Appearance clear
• Glu 1.4(2.5-5.0) 6.6
• Prot 1.76(0.15-0.4) 0.85
• Chl 129(120-130) 134
• AFB smear -negative
• Indian Ink- negative

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Progress................

• Anti -TB started EHRZ
• drug induced hepatitis (D7), stopped and
  challenged after ALT normalised
• T.prednisolone 50mg
• IVIG 5/7

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More results........

• ANFDsDNA positive
• C3C4- low
• APCR negative
• lupus anticoagulant negative
• TFT normal

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Progress

• general condition improved
• MMSE- improved
• Afebrile
• Liver enzymes- normalized
• Blood parameters improved
• Waiting for renal real biopsy

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But........................

• Persistent resting tachycardia.........
• Denied chest pain
• HRgt120/min
• ECG sinus tachy
• Afebrile
• P/E
• Presence of murmur
• PSM grade 3/5 best heard at lower Lt sternal
  edge, radiating to axilla

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• Urgent referral made to IJN.

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2 D echo ( IJN 26/11/ 04)

• LV was not dilated with on IDd of 4.9 cm and IDs
  of 2.9 cm.
• E/F- 62 .
• no RWMA
• LA dilated - 4.2 cm,
• mitral valve prolapse in the anterior leaflet
  of the mitral valve
• corresponded to MR which was eccentric radiating
  to the posterior wall of the LA.
• PA was mildly dilated.

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• Mild TR - pressure gradient of 28 mm hg,estimated
  PA pressure 45-50 mmhg.- mild pulmonary
  hypertension.
• RV was contracting well.
• mild pericardial effusion.
• fairly large vegetation measuring 0.5 -1 cm in
  the mitral leaflet in the atrial position.
• It was not very mobile -? Libman -sacks vegetation

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Video clip
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Earlier echo finding

• Good LV function
• EF 75
• No chamber enlargement
• No pericardial eff.
• Valves normal
• No vegetation

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Cardiovascular involvement.

• Resting tachycardia
• Murmur
• Elevated cardiacenzymes
• Echo findings

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Progress.

• Prednisolone cont 1mg/kg
• beta blockers metoprolol 25mg bd

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Latest review.....................

• 17th Jan 05
• Well
• No sm of active disease
• Seen by IJN
• NYHA FC 1
• severe MR with prolapsed AMVL with
  vegetation
• KIV for valve replacement

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Summary..

• 18/M/M
• SLE Nov 2004
• malar rash, oral ulcers, discoid lupus,
  vasculitic ulcers over both LL,
• haematological involvement (lymphopenia and
  pancytopenia )
• Renal ( awaiting bx report)
• ANA, anti-ds DNA ve, antimicrosomal Ab ve

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And..

• myocarditis and Libman-Sacks endocarditis with
  MVP and severe MR

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• Presumptive dx of TB meningitis ( Stage II)
• MRI findings and high CSF protein
• CSF for AFB was negative
• 6/12 intensive phase, followed by 6/12
  maintenance
• repeat MRI brain on 31/1/05
• Rt parietal lobe infarct with Lt hemiparesis
• ?vasculitis
• ?associated antiphospholipid syndrome,
• IgG ACA (2x) low titre

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Cardiovascular manifestation in SLE
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.every anatomical component of the heart

• Pericardium
• Myocardium
• Endocardium
• Valvular apparatus
• Coronary arteries

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• pericarditis,
• pericardial effusion,
• Libman-Sacks endocarditis,
• myocarditis,
• coronary artery disease,
• myocardial infarction.

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• left ventricular free wall rupture,
• acute mitral regurgitation following rupture of
  chordae tendinae
• aortic dissection

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Pericardial ds..

• Imaging / autopsy gt60 , clinically significant
  only in 30
• Most common
• Pericarditis at any stage
• Fluid elevated neutrophil count,
• elev protein
• low or normal glucose
• low complements
• Mild pericarditis (stable haemodynamically)
  NSAID
• Large per. (not responding to steroids)-
  drainage with window

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Cor. Art disease

• Cor. arteritis ischemic syndromes in SLE
• Distinction between CAD and arteritis
  angiographic studies more rapid change in
  luminal images
• CAD, - the detection of a single aneurysm on an
  angiogram may be compatible with diagnosis of
  atherosclerosis or vasculitis
• More evidence of vasculitis -demonstration of
  aneurysm formation followed by rapid stenosis

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• Despite young age atherosclerosis
• aetiopathogenesis of accelerated atherosclerosis
  - uncertain, but -multifactorial.
• Elevated lipid levels (especially early in the
  course of the disease)
• corticosteroid therapy ( increase lipid levels
  whereas antimalarials do the opposite)
• raised plasma homocysteine levels, and elevated
  antiphospholipid antibodies

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• reports of pathologically proven coronary artery
  vasculitis in serologically and clinically
  inactive patients
• coronary artery aneurysms have also been reported
  in SLE patients in the absence of detectable
  disease activity .

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Management

• Similar to routine artherosclerotic pt,
• Except arteritis aggressive treatment with
  steroid
• Thrombotic ds related to APLS long term high
  dose anti-coagulation

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Arrhythmias

• Tachyarr. D/t pericarditis or ischemia.
• Sinus tachy earliest manifestation of
  myocarditis
• Abnormal HR variability autonomic dysfunction/
  occult myocarditis
• Unexplained sinus tachy that resolves with Tx of
  SLE can occur in the presence of active SLE
  without cardiac involvement.

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Valves

• Common
• Systolic murmurs -1/3 lupus patients,
• diastolic murmurs -rather rare.
• The classic endocarditis described by Libman and
  Sachs (1924,) although identified in up to 50
  per cent of autopsied cases, rarely causes
  clinically significant lesions.
• Valvular thickening- striking feature
• Vegetation

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• Valvular insufficiency
• Histologically small vegetations (verrucae)
  comprising proliferating and degenerating valve
  tissue with fibrin and thrombi are seen.
• Located on the atrial side of the mitral valve
  and the arterial side of the aortic valve
• Immobile
• Rarely embolize and cause stroke
• Reported cases of valve replacement

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Khamashta at el 1990

• 132 prosp. echo
• prevalence - valvular 22.7
• adjacent to the edges of the mitral and aortic
  valves and - contain Ig and complement
  components, within the walls of the small
  junctional vessels in the active portions of the
  verrucous endocardial lesions.
• deposits might -represent immune complexes
  deposited via the circulation.
• these valve vegetations a/w antiphospholipid
  antibodies.

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• Mandell's 1987
• haemodynamically significant
• aortic incompetence and mitral regurgitation were
  the most frequently found
• Not well determined whether the most likely cause
  is a consequence of the underlying
  immunopathology of the disease or the
  predisposition to infection.
• reports of bacterial endocarditis in lupus
  patients do antedate corticosteroid therapy,
  suggesting that in some patients at least it is
  the primary immunopathology which predisposes to
  secondary bacterial infection.

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Myocarditis

• True myocardial involvement lt pericardial
  disease.
• Clinical myocarditis,
• unexplained tachycardia,
• congestive heart failure,
• arrythmias,
• prolongation of the PR interval on ECG
• cardiomegaly without pericardial effusion
• valvular disease,
• occurs 15 .

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• Histological - myocardium - mild non-specific
  perivascular infiltration with lymphocytes and
  neutrophils
• Intimal proliferation of the smaller
  intramyocardial arteries is also commonly
  reported, together with hyalinized vessels that
  may reflect either previous arteritis or primary
  thrombosis.

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Diagnosis..

• Clinical.
• 2-D echo ? RWM abnormality
• Other non invasive.
• Gallium scan

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Management..

• Urgent clinical attention
• Treatment strategies clinical experience, not
  trials
• Bed rest
• High dose steroids
• Monitoring response
• gallop rhythm, cardiomegaly,peripheral edema
  and ECG changes resolves

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