It’s the right time that primary care physician should be aware of

The thousand face of Lupus

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

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

Multi organ involvement

.

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

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

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

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 UlcersDiscoid lesions

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

“Serositis”

Inflammation of the membranes covering the lungs, heart and abdominal cavity

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

ACR Recommendations

Lupus Nephritis Classes

Class II (mesangial) Class III (focal proliferative) Class IV (diffuse proliferative)

Class V (membranous) Class VI (advanced sclerosis)

WHO Classification

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

Retinal involvement

Raynauds’ phenomenon

Vasculitis

Thrombosis

Other manifestations…

19

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

Does NOT mean you have lupus.

Good screening test- practically all patients with lupus. BUT not a specific test.

“Positive ANA”

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

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

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

Lupus Nephrit is Class III/IV (Init ial therapy)

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.

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

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

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

Care for lupus