A comprehensive review of Lupus for primary care physician and GPs

1. It’s the right time that primary care
physician should be aware of
The thousand face of Lupus

2.  Prevalence of SLE in the population:
20 to 150 cases per 100,000
 In women, prevalence rates vary from:
164 (Caucasian) per 100,000
to 406 (African American) per 100,000
Epidemiology

3.  Due to improved detection of mild disease, the
incidence nearly tripled in the last 40 years of the
20th century.
 Estimated incidence rates are 1 to 25 per
100,000 in North America, South America,
Europe and Asia.
Epidemiology

5. Multi organ involvement
.

6. Systemic Lupus Erythematosus
• Clinical symptoms related to the degree of
inflammation in various organs
• Skin and mucous membranes
• Synovium (joints)
• Serosal membranes
• Kidneys
• Central nervous system
• Lungs
• Heart
• Hematopoietic system

7. Signs and Symptoms
 Fatigue
 Headaches
 Painful or swollen joints
 Fever
 Anemia (low amounts of iron in the bloodstream)
 Swelling in feet, hands, and around eyes
 When breathing deep there is pain in the chest
 Photosensitivity
 Unusual hair loss
 Abnormal blood clotting
 Mouth or Nose Ulcers
 Fingers turning white or blue when cold
 Butterfly rash between nose and cheeks

8. Rheumatology e-dition, 4th Edition. Marc C. Hochberg, MD, MPH, Alan J. Silman, MD, Josef S. Smolen, MD, Michael E. Weinblatt, MD and Michael H. Weisman, MD
Malar Rash

9. Rheumatology e-dition, 4th Edition. Marc C. Hochberg, MD, MPH, Alan J. Silman, MD, Josef S. Smolen, MD, Michael E. Weinblatt, MD and Michael H. Weisman, MD
Cutaneous
Oral Ulcers
Discoid
lesions

10. Rheumatology e-dition, 4th Edition. Marc C. Hochberg, MD, MPH, Alan J. Silman, MD, Josef S. Smolen, MD, Michael E. Weinblatt, MD and Michael H. Weisman, MD
 Joint pain constitute
the most common
presenting
manifestation of SLE
Arthritis

11. “Serositis”
 Inflammation of the
membranes
covering the lungs,
heart and
abdominal cavity

12.  Involvement of the kidneys
during the course of the
disease occurs in up to 60%
of cases,
 resulting in worsening
morbidity and mortality.
Houssiau FA. Management of lupus nephritis: an update. J Am Soc Nephrol 2004;15:2694–704.
Lupus nephritis

13. ACR Recommendations

14. Lupus Nephritis Classes
Class II (mesangial) Class III (focal proliferative) Class IV (diffuse proliferative)
Class V (membranous) Class VI (advanced sclerosis)
WHO Classification

15. 19 Neuropsychiatric
Lupus syndromes
Central nervous system
Aseptic meningitis
Cerebrovascular disease
Demyelinating syndrome
Headache (including migraine and benign
intracranial hypertension)
Movement disorder (chorea)
Myelopathy
Seizure disorders
Acute confusional state
Anxiety disorder
Cognitive dysfunction
Mood disorder
Psychosis
Peripheral nervous system
Acute inflammatory demyelinating
polyradiculoneuropathy (Guillain-Barre´
syndrome)
Autonomic disorder
Mononeuropathy, single/multiplex
Myasthenia gravis
Neuropathy, cranial
Plexopathy
Polyneuropathy
The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum. 1999 Apr;42(4):599-608.

16. 16
Retinal involvement

17. Raynauds’ phenomenon
Vasculitis
Thrombosis
Other manifestations…

19. 19

21. Autoantibodies in SLE
• ANA
• Seen in 95% of SLE
• Not specific for SLE
• Seen in many
inflammatory,
infectious, and
neoplastic diseases
• Seen in 5% to 15% of
normal persons

22. Does NOT mean you have lupus.
Good screening test- practically all patients
with lupus. BUT not a specific test.
“Positive ANA”

23. Autoantibodies in SLE
• Anti-ds DNA
• Seen in 60% of patients with SLE
• Highly specific for SLE
• Low titer rarely seen in other inflammatory
conditions
• Strongest clinical association is with nephritis
• Anti-Sm (Smith)
• Seen in 10% to 30% of SLE patients
• Highly specific for SLE

27. When to Consider a Diagnosis of SLE
• Usually seen in women of childbearing age with:
• Constitutional symptoms of fever, weight loss,
malaise, and severe fatigue
• Skin rash and/or stomatitis
• Arthritis
• Renal disease
• Cytopenias
• Although 90% of patients are female, SLE can be
seen at any age in either sex

28.  No cure
 Treatment goal: relieve symptoms and protect organs
by decreasing inflammation and/or the level of
autoimmune activity in the body.
 Treatment options:
 Rest/Sleep
 Corticosteriods
 Hydroxychloroquine/Chloroquin (anti-malarial)
 Cytotoxic drugs (immunosuppressive medications)
 Biologic agents
Treatment

29. Lupus Nephritis Class III/IV
(Initial therapy)

30. MMF superior to Aza for maintenance?
 A total of 227 patients across sites in the US, Western
Europe, China, Argentina, and Mexico, who improved after
6 months of either high-dose CYC or MMF were randomly
assigned to maintenance treatment (116 to MMF and 111
to Aza)
 Over 3 years of follow up, MMF was statistically better than
AZA in time to treatment failure (a composite including
death, end-stage renal disease, doubling of serum
creatinine, and renal flare), and in each element of the
composite score.
 Severe adverse events occurred in significantly more
patients receiving AZA than receiving MMF.
Dooley et al. NEJM 2011;365:1886–95.

31. Leading cause of kidney disease, strokes and
heart disease in childbearing age women.
35-44 y/o lupus patients are 50 times more
likely to have a heart attack.
2X increased risk for carotid plaque
(atherosclerosis). (<40y/o)
Disability.
Morbidity
(1) Manzi S. et al. Am J Epidemiol. 1997;145;408-415. (2) Esdaile JM et al. Arthritis Rheum. 2001; 44 (10):2331-2337 (3) Roman MJ et al. N Engl J Med. 2003; 349: 2399-2406

32.  Over the past 50y, 5y-
survival in lupus pts
improved from 50% in
1955 to 82-95% in
1990s.
However, lupus patients
have a 3-5X increased
mortality compared to
the general population.
SLE pts in the 1980–1992 cohort.
Mortality
SLE pts in the 1950–1979 cohort. Pop=population; MN-W=Minnesota whites

33.  There is currently no certain way to prevent SLE
 But people who smoke may be more likely to
develop lupus.
 Avoiding smoking and perhaps other tobacco
products may decrease your risk of developing
lupus.
 UV light
 Physical & Psychological stressor
 Healthy life style
Prevention

43. Care
for
lupus