sle

1. SYSTEMIC LUPUS
ERYTHEMATOSUS:THE HYDRA
HEADED, CHAMELEONIC
DISEASE
Dr O. O. Adelowo, MBBS (Ib),FMCP,
FWACP,FACR,FRCP Edin FRCP Lond
Professor of Medicine/Consultant
Rheumatologist
Olabisi Onabanjo University
Teaching Hospital, Sagamu and Arthrimed
Specialist Clinic. Ikeja
Nigeria.
E-mail – femiadelowo2003@yahoo.com

2. INTRODUCTION
 Systemic Connective Tissue Disease
 Chronic multisystemic inflammatory disease
 Presents with features of acute and chronic
inflammatory processes
 Every and any organ
 Relapsing and Remitting Course

3. PREVALENCE
 Prevalence USA – 1:2000
 UK – 1:10,000 (USA)
 Annual incidence – 1:10,000 (USA)
 High prevalence African Americans
 Uncommonly reported Black Africans
 Symmons. Prevalent Gradient Theory
 NEW DATA SHOWS IT IS COMMON
 Sang-Cheol B et al. Arthritis Rheum. 1999.41:2091-2099
 Molokhia M et al. Lancet 2001:357:1414-1415
 Symmons DP. Lupus 1995:4:176-178
 McCarty DJ et al. . Arthritis Rheum. 1995:38:1260-1270
 Adelowo OO et al. Nigerian Medical Practitioner 1994;27:6-7
 Adelowo OO,Oguntona S Clinical Rheumatology 2009.28;6:699-703

4. Table 1 – SLE in Different Black
African Populations( Adapted from Bae,Fraser,Liaing Arthritis
Rheum.1999;41:2091-2099)
COUNTRY
NO OF CASES STUDY PERIOD ACR CRITERIA
GHANA
11 1983-1989 1971
GUINEA
1 1971 None
COTE D’ IVOIRE
9 1972-1983 1971
CONGO REPUBLIC 3 1961-1962 None
SENEGAL 4 1973-1975 None
ZIMBABWE 71 1962-1967 None
1970-1983 1971:1982

5. UGANDA 26 1968-1978 1971:None
KENYA 1 1961 None
SOUTH
AFRICA
187 1984-1990
1975-1987
1969-1975
1971
1982

6.  SEX: MOSTLY FEMALES (95%)
 FEMALES OF CHILD BEARING AGE
 AGE: 14-64.
 (Nigerian Population. Mean – 32 years)
 ELDERLY LUPUS IN UK

7.  Was a killer Disease
 Ten year survival now – 90%
 Improvement over past 30 years
– Earlier Diagnosis with ACR criteria
 Improvement medication
 Better treatment of infections
 Better management of complications
especially renal

8. MORTALITY/MORBIDITY
 Complications
 Nephritis and renal failure
 Infections
 Cardiovascular Diseases – Coronary
 Malignancy
 Atherosclerosis
 Medications

9. PATHOPHYSIOLOGY
 Autoimmune disease
 Immune Complex Disease
 Multisystemic microvascular inflammation
 Loss of T cell suppressor on B cell
 Overproduction of autoantibodies and immune
complexes
 Complement activation and consumption
 Disorder of immune complexes clearance
 Disordered Apoptosis

10. PATHOPHYSIOLOGY contd
 Deficient Thymus function
 Genetic
– High prevalence among monozygotic twins5
– -12% of relatives of patients develop SLE
– HLA DR 2, HLA DR 3
 Racial – commoner in Blacks than whites
 Hormonal – commoner in females
 Environmental – viruses, mycoplasma
 Block SK et al. Am J Med. 1975:59:533-52
 Lawrence JS et al. J Rheumatol. 1987: 299:515-18
 Figueroa JE, Densen P. Clin Microbiol Rev. 1991:4:359-95
 Arnett FC, Reveille JD. Rheum. Dis Clin North Am. 1992:38:110-14

11. Recurrent fever(Malaria-Typhoid-Malaria syndrome),polyarthralgia,weight loss,fatigue,loss of weight,seizures
ACR CRITERIA
1.Malar rash
2.Discoid rash
3.Photosensitivity
4.Oral and Photosensitivity
5.Non erosive arthritis
6.Pleuritis and pericarditis
7.Renal disorders
8.Seizures or Psychosis
9.Haematologic disorders
10.Immunologic disorders- Anti DNA,Anti Sm,ACA, False +ve VDRL
11.Positive ANA
Hochberg MC. Arthritis Rheum 1997;40:1725

12. CLINICAL FEATURES IN ORGANS
 CUTANEOUS
 Erythema bullous
 Butterfly rashes
 Subacute palpable erythematous
 Plagues associated with RO/SSA
 Discoid
 Alopecia
 Raynaud

13.  RENAL
 Lupus Nephritis-
Albuminuria(3+Dipstick);Urinary
deposits;Haematuria;Elevated Serum Urea: WHO
Classification
 Acute renal failure
 Nephrotic Syndrome
 Pyelonephritis
 Chronic Renal failure

14.  CENTRAL NERVOUS SYSTEM
 Headache
 Aseptic meningitis
 Cranial Neuropathy
 Peripheral neuropathy
 Blindness
 Seizures
 Encephalitis
 Cognitive impairment
 Depression
 Acute psychosis
 Acute Brain Syndrome

15.  PULMONARY
 Pneumonitis
 Pleurisy/pleural effusion
 Pulmonary embolism
 Pulmonary fibrosis
 Alveolar haemorrhage

16.  GASTRO INTESTINAL
 Dysphagia
 Mouth Ulcers
 Peritonitis
 Pancreatitis
 Mesenteric Vasculitis
 Bowel Infarction
 Auto immune Hepatitis

17.  CARDIOVASCULAR
 Hypertension
 Pericarditis/effusion
 Endocarditis (Libman-Sachs)
 Myocarditis
 Coronary artery disease
 RETICULO ENDOTHELIAL
 Lymphadenopathy
 Hepatosplenomegaly

18.  ANTIPHOSPHOLIPID SYNDROME:
 Recurrent Arterial or Venous
Thrombosis; Recurrent Pregnancy
Losses; Thrombocytopaenia; Positive ACA/
LAC
 Case Reports among Nigerians- ref
 Adelowo OO.Oguntona S. Antiphospholipid Syndrome:Report of Five Cases. East African
Medical Journal.2009;2:517-519

19. TABLE 2 CLINICAL FEATURES
AMONG 66 NIGERIAN SLE PATIENTS
(Adapted Adelowo OO,Oguntona S. Clin Rheumatol. 2009.28;6:699=703)
Clinical Features Number (%)
Polyarthralgia /arthritis 58(87)
Fever 33(50)
Hair loss 32(45)
Discoid rashes 29(43.9)
Weight loss 28(42.4)
Mouth/pharyngeal ulcers 22(33)
Fatigue 18(27.3)
Pleuritic chest pain 17(25.8)
Malar rashes 14(21.2)
Headache/migraine 22(33.3)
Seizures 14(21.2)
Photosensitivity 6(9)
Cognitive Impairment 8(12.1)
Pedal oedema 4(6.1)

20. DIFFERENTIAL DIAGNOSIS
 Fibromyalgia
 Rheumatoid Arthritis
 Hepatitis C
 MCTD
 Benign Hypermobility Syndrome
 Drug Induced SLE – Procainamide,
Hydralazine, Isoniazid, Methyldopa,
Chlorpromazine.

21. SEROLOGY MARKERS
 ANA sensitive but not specific – 95%
 Different staining patterns – Homogeneous, Speckled, Rim,
Nucleolar
 Anti-Sm – specific for SLE
 Anti-double stranded DNA – specific but not sensitive
 Single stranded DNA in drug induced SLE
 Anti RNP suggestive of MCTD but present in black SLE
 Anti Ro/SSA, La/SSB – neonatal lupus and congenital heart
block
 Anti-Ribosomal P-lupus- Cerebritis
 ACA/LAC – Anti phospholipid Syndrome
 ESR – markedly elevated (In Nigerian patients. Above 100).
 CRP – usually normal except infections

22. TABLE 3: SEROLOGY MARKERS IN
NIGERIAN SLE PATIENTS
(Adapted Adelowo OO, Oguntona S Clin Rheumatol.2009;28:699-703)
SEROLOGY NUMBER TESTED POSITIVE (%)
Rheumatoid factor 21 5(23.8)
Anti CCP 4 0
ANA 66 65(98.5)
Anti ds DNA 26 14(53.8)
ENA 21 15
Anti Sm 11 7(63.6)
Anti RNP 15 10(66.7)
Anti Ro 15 7(46.7)
Anti La 12 1(9.0)

23. TABLE 4:ANA TITRES AMONG
NIGERIAN PATIENTS
ANA TITRE FREQUENCY (%)
1:40 1(1.5)
1:80 8(12.3)
1:160 11(16.9)
1:320 4(6.3)
1:640 12(18.5)
1:1280 8(12.3)
1:2560 11(16.9)
1:5120 6(9.2)
1:102,400 1(1.5)
Titre not stated 3(4.6)
Speckled pattern 75.9%
Homogenous 24.1%

24. MANAGEMENT
 NSAIDS for joint involvement only.
 Steroid cream for skin involvement only
 Hydroxychloroquine for skin, musculoskeletal involvement
 Corticosteroids for skin, musculoskeletal, and other systems.
 Immunosuppressives for severe SLE, and CNS, Renal,
Pulmonary and other systems
 Corticosteroids mainstay-Oral, Systemic,Intra articular


25. DRUGS
 A. Antimalarials- Hydroxylchloroquine
 B. IMMUNOSUPPRESSIVES
– Azathioprine
– Cyclophosphamide
– Methotrexate
– Cyclosporine
– Mycophenolate mofetil
– Bromocriptine
– Thalidomide for severe skin manifestations
– Dapsone for severe skin manifestations
– Fludarabine
– Biologics – Etanercept, Infliximab, Rituximab
 6. Plasmapharesis
 7. Intravenous Immunoglobulins (IVIG)

26. THANK YOU