Dr Fariz Yahya

Rheumatology Unit

UMMC

Case Report

Miss RP 13 yr old with underlying SLE SLE diagnosed 2009 Eldest of 3 siblings Father taxi driver, mother factory worker

Case Report

Initially presented with systemic symptoms 10/2009

Fever Arthralgia Bicytopenia

Hb 5.5, WBC 6.3, plt 96

AIHA: Coombs positive with evidence of haemolysis

Case Report ESR 140 Low C3 (14),C4 (<10) Raised IgG Anticardiolipin – 21 Lupus anticoagulant: + ANA 1:1280, dsDNA 692 Serositis : bilateral pleural effusions and

ascites UFEME: NAD ECHO: normal

Initial ECG in 2009

Case Report

BMAT no evidence of malignancy received IVI Methylprednisolone

4mg/kg/day responded to steroids discharged with oral steroids

tapering doses

started Azathioprine 11/2009 added Hydroxychloroquine 2/2010

Case Report

However, ESR remained high considered for Rituximab Appeared Cushingoid BMD in 06/2011 - osteoporotic for her

age

ESR trend

Recent admission

Presented again on 07/2011 with AIHA Hb was 5.7, Plt 296, ESR 140, CRP 3.6 dsDNA 237 Hapto 59, se iron 3.5, ferritin 1252 Raised LDH 314 and Retics 14.9% IVI Methylprednisolone given Hb improved to 7.3 Azathioprine changed to Mycophenolate

Recent admission

Developed left sided chest pain after discharge

Atypical, tender left chest wall No fever ECG: Q waves and inverted T in II, III,

aVF, V5-V6Dynamic changes

Trop I was 8.36,Trop T 0.46, CK 464, CKMB 4

ECGs

ECGs

ECGs

ECGs

Case Report

ECHO: Normal LVEF 63%, no RWMA, LA size normalPASP 38mmHgNo pericardial effusionNo pericarditis

Multi slice CT coronary angiogram: normal

CTPA: No Pulmonary Embolism

Further follow up

Treated as Myocarditis Given NSAIDs, aspirin Symptoms improve No further chest pain

Latest ECG

Discussion

Possible differential diagnosis?

Differential diagnosis

Myocarditis Pericarditis Valvular disorders Coronary artery disease Costochondritis Anaemia induced Pulmonary embolism

Differential diagnosis

Small vessel vasculitis Pleurisy Drug induced: eg prednisolone

Myocarditis

Not common: 3-14% of patients can develop myocarditis (Routray et al 2004)

Chest pain, palpitations or SOB Resting tachycardia Can have either minimal symptoms or

CCF if severe

Myocarditis

Non-specific ST/T wave changes on ECG Conduction disturbances and heart block Common Echo findings include decreased

LV ejection fraction and segmental wall motion abnormalities (Law et al, 2005) or diffuse hypokinesia

Myocarditis

Endomyocardial biopsy remains technique of choice for diagnosis (Tincani et al, 2006)Invasive and subject to sampling error

Patients improve with cardiac support, steroids and immunosupression eg Azathioprine, Cyclophosphamide, or IVIG

Improve in symptoms and LVEF

Pericarditits

Most common cardiac abnormality in SLE: 6 – 45%

Left shoulder pain precipitated by lying down, relieved by sitting up

Consider any cause: unknown, infection, radiation, trauma, drugs, metabolic, malignancy, IBD

Any 2: chest pain, ECG changes, pericardial friction rub, pericardial effusion

ECG in pericarditis

ECG in pericarditis ECG showing diffuse upsloping ST segment

elevations seen best here in leads II, III, aVF, and V2 to V6. There is also subtle PR segment deviation (positive in aVR, negative in most other leads). ST segment elevation is due to a ventricular current of injury associated with epicardial inflammation; similarly, the PR segment changes are due to an atrial current of injury which, in pericarditis, typically displaces the PR segment upward in lead aVR and downward in most other leads.

Courtesy of Ary Goldberger, MD.

Pericarditis

Treat with aspirin and NSAIDS Some studies suggest use of colchicine

or prednisolone

Valvular disease Systolic murmur in 16-44% of pts Mitral valve involvement is most common

(Mitral regurg) May occur at any time and unrelated to

disease activity Vegetation or thickening more related to

APLS in SLE: Anti-cardiolipin antibody

Libman Sacks endocarditis Libman-sacks endocarditis

Verrucous endocarditis of valve leaflet, papillary muscles and mural endocardium

The verrucae are near the edge of the valve

Consists of immune complexes, mononuclear cells, haematoxylin bodies, fibrin and platelet thrombi

Healing leads to fibrosis, scarring or calcification

Verrucous endocarditis with valvular vegetations (arrows) in a 52-year-old woman with systemic lupus erythematosus who died of pneumonia and chronic interstitial pneumonitis. The vegetations had not been observed by echocardiography, although a cardiac murmur had been heard by auscultation.

Libman Sacks endocarditis Usually aymptomatic If lesions are extensive: can produce

valve deformity – MR/AR Verrucae can fragment and produce

emboli – leading to IE

Coronay artery disease

Role of autoimmunity in atherosclerosis Increase cardiac and cerebrovascular

events in pts with autoimmune diseases (Salmon et al, 2001)

Risk of developing CAD is 4-8 times higher in SLE pts

Consider risk factors

Summary

Generally cardiopulmonary problems associated with SLE respond to treatment

Treatment must be tailored to each patient and problem