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-V6

Dynamic changes

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

ECGs

ECGs

ECGs

ECGs

Case Report

ECHO:
Normal LV
EF 63%, no RWMA, LA size normal PASP 38mmHg No pericardial effusion

No 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