the problem of lupus
DESCRIPTION
Dr Evelyn Salido talks about the burden of disease of SLETRANSCRIPT
THE PROBLEM OF LUPUS
Evelyn Osio-Salido, MD, FPCP, FPRA LMLR 2014
UP-PGH Medical Center
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
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
DIAGNOSIS: DIVERSITY OF MANIFESTATIONS
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
Diagnosis by classification criteria 1997 update of the 1982 American College of Rheumatology criteria
Petri M. Arthritis & Rheumatism. Aug 2012
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.
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
Damage
Ocular Neuropsychiatric Renal
Pulmonary Cardiovascular Peripheral vascular
Gastrointestinal Musculoskeletal Skin
www.rheumatology.org
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
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
Chronic Illness Lupus Flares
Organ Damage
Athero sclerosis
Side Effects of Drugs
Life challenges Education, Work, Marriage, Family,
Community
Treatment
Lupus Flares
Organ Damage
Athero sclerosis
Side Effects of
Drugs
Life challenges Education, Work, Marriage, Family,
Community
• Multifaceted & holistic • Individualized • Well-monitored
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
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
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
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
The cause: Pathogenesis of SLE
SLE: A Primer for managed care. www.ajmc.com/publications/supplement/2012/SLE-Primer-for-managed-care/May12
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
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
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
SLE: A Primer for managed care. www.ajmc.com/publications/supplement/2012/SLE-Primer-for-managed-care/May12
SLE: A Primer for managed care. www.ajmc.com/publications/supplement/2012/SLE-Primer-for-managed-care/May12
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