29
THE PROBLEM OF LUPUS Evelyn Osio-Salido, MD, FPCP, FPRA LMLR 2014 UP-PGH Medical Center

The Problem of Lupus

Embed Size (px)

DESCRIPTION

Dr Evelyn Salido talks about the burden of disease of SLE

Citation preview

Page 1: The Problem of Lupus

THE PROBLEM OF LUPUS

Evelyn Osio-Salido, MD, FPCP, FPRA LMLR 2014

UP-PGH Medical Center

Page 2: The Problem of Lupus

The Name

Lupus (n.), Medieval Latin, late 14c • Apparently because it "devours" the affected part • Diseases that cause ulcerations of the skin • Earliest known medical use of “lupus”� appeared in a 10th

century biography of St. Martin (lived in 4th-century Gaul) The Bishop of Liege was healed at St. Martin's shrine in Tours:

“He  was  seriously  afflicted  and  almost  brought  to  the  point  of  death  by  the  disease  called  lupus.  The  location  of  the  disease  was  not  to  be  seen,  nonetheless,  a  sor<  of  thin  red  line  remained  as  a  mark  of  the  scar.”  

Wallace, Daniel J.; Hahn, Bevra Hannah, 2007. Dubois' Lupus Erythematosus, 7th Edition

Page 3: The Problem of Lupus

History •  “Lupus” associated with cancer or tuberculosis. •  Diagnosis & treatment of LE in the domain of dermatologists until 1940s.

•  SLE was recognized by its cutaneous findings •  visceral manifestations were considered secondary to the cutaneous •  In 1940s, accepted that SLE may occur without skin lesions

•  The pioneers of rheumatology (the late 1920s) had neither technical nor therapeutic superiority over other internists. •  1948- rheumatoid factor and the LE cell (increase diagnosis of SLE) •  1949- cortisone therapy (patients can be helped, treatment required specialized

knowledge) •  1950- establishment of the Institute of Arthritis and Metabolic Diseases in the National

Institutes of Health.

•  Shift in interest in LE from clinical description to immunologic research led to continued intensification of scientific interest (Index Medicus/Medline) •  8 columns in 1960, 21 in 1982, 25 in 1987, 31 in 1992, 47 in 1997, thousands

of references in 2006, 60,000 in 2014.

Wallace, Daniel J.; Hahn, Bevra Hannah, 2007. Dubois' Lupus Erythematosus, 7th Edition

Page 4: The Problem of Lupus

DIAGNOSIS: DIVERSITY OF MANIFESTATIONS

Page 5: The Problem of Lupus

Diagnosis: other syndromes At onset e.g. ITP

Silent bystander or mimic

Concurrent e.g. APAS

Overlap e.g. RA

Concurrent e.g.

Hashimoto’s thyroiditis

Concurrent e.g.

Infection

Drug effects

Page 6: The Problem of Lupus

Diagnosis by classification criteria 1997 update of the 1982 American College of Rheumatology criteria

Page 7: The Problem of Lupus
Page 8: The Problem of Lupus
Page 9: The Problem of Lupus
Page 10: The Problem of Lupus

Petri M. Arthritis & Rheumatism. Aug 2012

Page 11: The Problem of Lupus

Diagnosis: no single test ANA

•  Immunofluorescence is the standard approach • Staining patterns (i.e., homogeneous or diffuse, speckled, rim, nucleolar, or centromere)

•  Depend on the location of the target antigen •  Autoantibodies against different nuclear antigens

•  Ideal screening test •  Simple •  High sensitivity (95% when human cultured cells used as substrate) •  Negative test- <3% chance of SLE; not R/O (+) typical features

•  Low specificity •  Positive in Scleroderma, PM-DM, RA, autoimmune thyroiditis,

autoimmune hepatitis, infections, neoplasms, & many drugs •  Healthy individuals:10-35% of individuals > 65 yrs ; low titer (<1:40)

Firestein. Kelley's Textbook of Rheumatology, 8th ed. 2008.

Page 12: The Problem of Lupus

Disease: acute & chronic Active disease vs organ damage

Irreversible

Not due to active disease

Since onset of lupus

Present for at least 6 months

Ascertained by clinical assessment

Repeat episodes 6 months apart to be scored 2

Same lesion cannot be scored twice

www.rheumatology.org

Page 13: The Problem of Lupus

Damage

Ocular Neuropsychiatric Renal

Pulmonary Cardiovascular Peripheral vascular

Gastrointestinal Musculoskeletal Skin

www.rheumatology.org

Page 14: The Problem of Lupus

Skin and others  Scarring chronic alopecia   1  Extensive scarring or panniculum other than scalp and pulp space   1  

Skin ulceration (excluding thrombosis) for > 6 months   1  

Premature gonadal failure   1  Diabetes (regardless of treatment)   1  Malignancy (exclude dysplasia), score 2 if > 1 site   1 (2)  

www.rheumatology.org

Page 15: The Problem of Lupus

Neuropsychiatric  Cognitive impairment (e.g. memory deficit, difficulty with calculation, poor concentration, difficulty in spoken or written language, impaired performance levels) or major psychosis  

1  

Seizures requiring therapy for 6 months   1  

Cerebrovascular accident ever (score 2 if > 1)   1 (2)  Cranial or peripheral neuropathy (not optic)   1  

Transverse myelitis   1  

Renal  Estimated or measured GFR<50%   1  

Proteinuria ≥3.5 gm/24hours   1  

ESRD (+/- dialysis or transplantation)   3  

www.rheumatology.org

Page 16: The Problem of Lupus

Chronic Illness Lupus Flares

Organ Damage

Athero sclerosis

Side Effects of Drugs

Life challenges Education, Work, Marriage, Family,

Community

Page 17: The Problem of Lupus

Treatment

Lupus Flares

Organ Damage

Athero sclerosis

Side Effects of

Drugs

Life challenges Education, Work, Marriage, Family,

Community

•  Multifaceted & holistic •  Individualized •  Well-monitored

Page 18: The Problem of Lupus
Page 19: The Problem of Lupus

The management of SLE should be based on shared decisions between the informed patient and her/his physician.

Treatment of SLE should aim at ensuring long-term survival, preventing organ damage, and optimising health-related QOL, by controlling disease activity and minimising comorbidities and drug toxicity.

The management of SLE requires an understanding of its many aspects and manifestations, which may have to be targeted in a multidisciplinary manner.

Patients with SLE need regular long-term monitoring and review and/or adjustment of therapy.

T2T in SLE: overarching principles

van Vollenhoven RF, et al. Ann Rheum Dis 2014;73:958–967. doi:10.1136/annrheumdis-2013-205139

Page 20: The Problem of Lupus

T2T in SLE: recommendations 1. The treatment target of SLE should be remission of systemic symptoms and organ manifestations or, where remission cannot be reached, the lowest possible disease activity, measured by a validated lupus activity index and/or by organ-specific markers. 2. Prevention of flares (especially severe flares) is a realistic target in SLE and should be a therapeutic goal. 3. It is not recommended that the treatment in clinically asymptomatic patients be escalated based solely on stable or persistent serological activity. 4. Since damage predicts subsequent damage and death, prevention of damage accrual should be a major therapeutic goal in SLE.

van Vollenhoven RF, et al. Ann Rheum Dis 2014;73:958–967. doi:10.1136/annrheumdis-2013-205139

Page 21: The Problem of Lupus

T2T in SLE: recommendations 5. Factors negatively influencing health-related quality of life (HRQOL), such as fatigue, pain and depression should be addressed, in addition to control of disease activity and prevention of damage. 6. Early recognition and treatment of renal involvement in lupus patients is strongly recommended. 7. For lupus nephritis, following induction therapy, at least 3 years of immunosuppressive maintenance treatment is recommended to optimise outcomes. 8. Lupus maintenance treatment should aim for the lowest glucocorticoid dosage needed to control disease, and if possible,glucocorticoids should be withdrawn completely. van Vollenhoven RF, et al. Ann Rheum Dis 2014;73:958–967. doi:10.1136/annrheumdis-2013-205139

Page 22: The Problem of Lupus

T2T in SLE: recommendations 9. Prevention and treatment of antiphospholipid syndrome (APS)-related morbidity should be a therapeutic goal in SLE; therapeutic recommendations do not differ from those in primary APS. 10. Irrespective of the use of other treatments, serious consideration should be given to the use of antimalarials. 11. Relevant therapies adjunctive to any immunomodulation should be considered to control comorbidity in SLE patients.

van Vollenhoven RF, et al. Ann Rheum Dis 2014;73:958–967. doi:10.1136/annrheumdis-2013-205139

Page 23: The Problem of Lupus

The cause: Pathogenesis of SLE

SLE: A Primer for managed care. www.ajmc.com/publications/supplement/2012/SLE-Primer-for-managed-care/May12

Page 24: The Problem of Lupus

  PROBLEM   SOLUTION  NAME   Sounds frightening   Get to know it  

DIAGNOSIS   Difficult, often delayed   Thorough history and PE, analysis of clinical course  

TREATMENT   Complicated   Complete problem list  

EFFECTS ON THE INDIVIDUAL  

Extensive & lifelong   Awareness, Counseling  

CAUSE   Not fully elucidated   Do/share/keep up with research  

Summary

Page 25: The Problem of Lupus

Figure 1 Map to demonstrate the global prevalence of autoimmune disease

Shoenfeld N et al. (2009) The effect of melanism and vitamin D synthesis on the incidence of autoimmune disease

Nat Clin Pract Rheumatol doi:10.1038/ncprheum0989

Page 26: The Problem of Lupus

Salido E & Reyes B. Lupus 2010. SLE: A Primer for managed care. www.ajmc.com/publications/supplement/2012/SLE-Primer-for-managed-care/May12

Prevalence of SLE from 15 countries (Eastern, Southeastern, Western Asia)

30-50 (70)/100,000 population

US Population, 2012   Incidence per 100,000 person-years  

African American women   9.2   Caucasian women   3.5   African American men   0.7   Caucasian men   0.4  Asian population   Incidence per 100,000 population   Hong Kong, 2006 Overall Females  

2.8 5.1  

India   1,000   Japan   0.9  

Incidence of SLE

Page 27: The Problem of Lupus

SLE: A Primer for managed care. www.ajmc.com/publications/supplement/2012/SLE-Primer-for-managed-care/May12

Page 28: The Problem of Lupus

SLE: A Primer for managed care. www.ajmc.com/publications/supplement/2012/SLE-Primer-for-managed-care/May12

Page 29: The Problem of Lupus

THANK YOU