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
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
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
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
UGANDA 26 1968-1978 1971:None
KENYA 1 1961 None
SOUTH
AFRICA
187 1984-1990
1975-1987
1969-1975
1971
1982
SEX: MOSTLY FEMALES (95%)
FEMALES OF CHILD BEARING AGE
AGE: 14-64.
(Nigerian Population. Mean 32 years)
ELDERLY LUPUS IN UK
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
MORTALITY/MORBIDITY
Complications
Nephritis and renal failure
Infections
Cardiovascular Diseases Coronary
Malignancy
Atherosclerosis
Medications
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
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
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
CLINICAL FEATURES IN ORGANS
CUTANEOUS
Erythema bullous
Butterfly rashes
Subacute palpable erythematous
Plagues associated with RO/SSA
Discoid
Alopecia
Raynaud
RENAL
Lupus Nephritis-
Albuminuria(3+Dipstick);Urinary
deposits;Haematuria;Elevated Serum Urea: WHO
Classification
Acute renal failure
Nephrotic Syndrome
Pyelonephritis
Chronic Renal failure
CENTRAL NERVOUS SYSTEM
Headache
Aseptic meningitis
Cranial Neuropathy
Peripheral neuropathy
Blindness
Seizures
Encephalitis
Cognitive impairment
Depression
Acute psychosis
Acute Brain Syndrome
PULMONARY
Pneumonitis
Pleurisy/pleural effusion
Pulmonary embolism
Pulmonary fibrosis
Alveolar haemorrhage
GASTRO INTESTINAL
Dysphagia
Mouth Ulcers
Peritonitis
Pancreatitis
Mesenteric Vasculitis
Bowel Infarction
Auto immune Hepatitis
CARDIOVASCULAR
Hypertension
Pericarditis/effusion
Endocarditis (Libman-Sachs)
Myocarditis
Coronary artery disease
RETICULO ENDOTHELIAL
Lymphadenopathy
Hepatosplenomegaly
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


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

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)


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