Management of Systemic Lupus Erythematosus(SLE) and brief literature review.

2. OBJECTIVES
 Case Presentation
 Definition
 Epidemiology and Etiology
 Pathology
 Management – Hx, PE, Work-up, DD, Rx
 Prognosis

3. CASE PRESENTATION

4. HISTORY
 PC: Generalized body weakness x3/7
 Dizziness x 3/7
 HPC: Sxx began 3days ago and have worsened
prgressively. Visited the A. Polyclinic where she was
referred here for further mngt.
 ODQ: chills+, fevero, dysgeusiao, cougho, dysuriao,
vaginal dischargeo, bleeding PRo, hematemesiso,
arthralgia+, skin rash+, vomitingo,diarrheao,
headacheo hx of jaundiceo, wt losso, dysphagia+,
paraesthesia+

5.  DH: Nil of Note
 SH: Lives at Ashaiman, ROHo, Smokingo, NHISo

6. P.E.
 Middle-aged woman looking unwell
 Afebrile, Anicteric, Pallor++, mildly dehydrated,
bipedal edema, L+(submandibular)
 CVS: S1S2Mo BP 110/70 Pules 120bpm RGV
 Chest: Clinically clear SpO2- 98%
 Abd: soft, MWR, non-tender, L(2cm, non-
tender)So2Ko
 CNS:GCS- 15/15, Power 5/5 in all limbs

7. Status Localis
 Ulnar deviation of both digiti minimi
 Flattening of both thenar and hypothenar
eminences
 Pt unable to open mouth, oral ulcers, but no
microstomia
 Hyperpigmented macules – trunk and Upper Limbs,
Malar Rash on face

8. DIAGNOSIS/PLAN
 Diagnosis = Symptomatic Anaemia 2o ?cause
 PLAN
 Urgent FBC, GXM x2units of PRBCs
 IVFs – 1L N/S, 1L R/L
 Blood film comment
 Tb folic acid 5mg dly x 30
 Tb Fersolate 200mg tds x 30/7

9. Labs
 Hb – 6.7g/dl
 WBC – 1.8 x 109/l
 Neu – 0.32 x 109(18.1%)
 Lym(0.94x109(52.5%)
 Plt – 71x109/l
 HCT – 21.5%
 RDW – 20.6%
 MCV – 79/um3
 MCH – 24.6pg
 MCHC 31.3g/dl

10.  Impression: Pancytopenia 20 ?cause
 Differential – Autoimmune disorder r/o SLE and RVI

11. PLAN
 ANA, ds-dNA, BUE & Cr, LFT
 Hemotransfuse 2units of whole blood
 IVF 3.5L – 2L N?S, 1L 5% Dex, 500ml R/L
 IV Hydrocortisone 200mg 8hrly x 24hrs
 Admit to GFW

12. Day 2-5
 Same Mngt
 Yet to do labs
 Day 5 – Able to swallow
 Stop Hydrocortisone
 Tb Prednisolone 40mg dly x 14/7
 Tb Omeprazole 20mg bd x 5/7

13.  VCT – non-reactive
 ESR – 55(0-20)
 Urea (6.37(2.1-7.1), Cr- 136.7(53-123.8)
eGFR -48(90-120)
 Na – 135.9(136-150), K – 4.07(3.5- 5.5)
 Total Protein – 50.4(60-80), Albumin13.6(30-55)
 AST- 193(5-40), ALT -62(10-40), ALP -353.2(5-270)
 Total bil 0- 6.41, Direct bil – 3.15, Indirect bil – 3.26

14. Day 6
 c/o – diarrhea and vomiting
 Tabs Cipro and Flagyl added
 Awaiting ANA and ds- DNA(samples to be sent to
S/A)

15. LITERATURE REVIEW

16. DEFINITION
 SLE is a chronic inflammatory, multisystemic,
autoimmune disease in which organs and cells
undergo damage mediated by tissue-binding
autoantibodies and immune complexes

17. EPIDEMIOLOGY/ETIOLOGY
 M:F ratio – 1:9
 Peak age of 20-40yrs
 All ethnic groups affected but commoner in African
Americans(>>Africans)
 Accounts for 5.28% rheumatology cases in
Nigeria(Adelowo, O.O. and Oguntona S.A.)
 Etiology is from a complex interplay of genetic and
environmental factors(drugs)

18. PATHOLOGY
 Widespread vasculitis from fibrinoid and immune
complex deposition on capillaries, arterioles and
venules of vital organs and serous membranes.

19. MANAGEMENT

20. HISTORY
 Easy fatiguability, malaise, symptoms of anemia,
arthralgia(small jts in 90% of pts), Raynaud’s phenomenon,
gangrene of the digits
 Skin Rash – malar(butterfly) rash, livedo reticularis,
alopecia, photosensitivity
 Edema(renal involvement), fever, lymphadenopathy
 Gum bleeding, menorrhagia, purpura (thrombocytopenia)
 Cough, pleuritic chest pain
 Neuropsychiatric Sxx, seizures
 PMH – HPT, Prev. episodes(remissions and exacerbations)
 DH: hydralazine, procainamide, minocycline,

21. Drug History
 Antiarrhythmics - procainamide, disopyramide, and propafenone;
 Antihypertensives - hydralazine; methyldopa, several angiotensin-
converting enzyme inhibitors and beta blockers
 Antithyroid – Propylthiouracil
 Antipsychotics - Chlorpromazine and lithium;
 Anticonvulsants - carbamazepine and phenytoin;
 Antibiotics - isoniazid, minocycline, and macrodantin;
 Antirheumatic - sulfasalazine; penicillamine
 Diuretic - hydrochlorothiazide;
 Antihyperlipidemics - lovastatin and simvastatin;
 Interferons and TNF inhibitors.

22. P.E.
 General Exam – Fever, Jaundice(rarely autoimmune
hepatitis), Pallor, edema, lymphadenopathy
 Mouth ulcers, alopecia, livedo reticularis, discoid patches
 Sun-exposed areas, elbows and knees - vasculitic rashes
and subcutaneous nodules
 Nails – splinter hemorrhages, nail-fold capillaries and
periungual infarcts
 Hands - Palmar erythema, Raynaud's phenomenon,
arthritis

23. Malar rash

24. Discoid Rash

25. Livedo Reticularis

26. Subcutaneous Nodules

27. Systemic Exam
 CVS – HPT, Murmurs(Libman-Sacks endocarditis),
pericardial rub(pericarditis)
 RS – decr AE, pleural rub, Creps – r/o Pleural
Effusion, Atelectasis, pulmonary fibrosis
 CNS – Decre orientation – r/o Seizures, cerebellar
ataxia, aseptic meningitis, cranial nerve lesions,
cerebrovascular disease or a polyneuropathy
 Eyes - hard exudates, and haemorrhages,
episcleritis, conjunctivitis or optic neuritis, but
blindness is uncommon

28. Work-up
 FBC – leucopenia, lymphopenia and/or
thrombocytopenia. An autoimmune haemolytic
anaemia occurs. The ESR is raised in proportion to
the disease activity. CRP is normal.
 ANA, anti-dsDNA, Anit-sm, antihistones
 BUE & Cr
 Urine R/E - proteinuria, cellular casts
 Head CT scan/MRI – infarcts, hemorrhages and
cerebral atrophy

29. Diagnostic criteria
 1. Malar rash
 2. Discoid rash
 3. Photosensitivity
 4. Oral ulcers
 5. Arthritis - nonerosive arthritis
 6. Serositis - pleuritis, pericarditis
 7. Renal involvement
 8. Neurological involvement - seizures, psychosis
 9. Haematological involvement
 10. Antinuclear antibody - Anti-dsDNA, anti-Sm, and/or anti-phospholipid
 11. Immunological disorder - An abnormal titer of ANA

30. Differentials
Drug-induced lupus
Antiphospholipid syndrome
Mixed Connective Tissue disease
CREST Syndrome
Scleroderma
Sjogren Syndrome
Polymyositis
Acute Pericarditis, Infective Endocarditis, Rheumatoid
Arthritis
Lyme Disease

31. Treatment
 Corticosteroids
 NSAIDS
 Antimalarials
 Immunomodulators –Cyclophosphamide,
Methotrexate, Azathioprine, Mycophenolate
 Anti-CD20 monoclonal antibodies eg. Rituximab
 Avoid exposure to sunlight
 Encourage patient to join a lupus society

32. Prognosis
 An episodic course is characteristic, with
exacerbations and complete remissions that may
last for long periods
 The arthritis is usually intermittent but arthralagia
and fatigue are often more persistent
 10-year survival rate is about 90%, although much
lower if major organ-based complications are
present

33. QUESTIONS???