Systemic lupus erythematosus and its management

An inflammatory disease caused when the immune system attacks its own tissues.
Lupus (SLE) can affect the joints, skin, kidneys, blood cells, brain, heart and lungs.
Symptoms vary but can include fatigue, joint pain, rash and fever. These can periodically get worse (flare up) and then improve.
While there is no cure for lupus, current treatments focus on improving quality of life through controlling symptoms and minimizing flare-ups. This begins with lifestyle modifications, including sun protection and diet. Further disease management includes medication such as anti-inflammatories, steroids, and immunosuppressives.

1. Systemic Lupus Erythematosus (SLE) Sachin Dwivedi Clinical Instructor College of Nursing, AIIMS Rishikesh

2. INTRODUCTION Systemic lupus erythematosus is a chronic, multisystem, inflammatory, autoimmune disorder characterized by formation of autoantibodies directed against self-antigens and immune-complex formation resulting in damage to essentially any organ.

3. INTRODUCTION Lupus is the Latin word for wolf. Erythematosus means red rashes. In 1851, Dr. Cazenave discovered red rashes on a patient’s face that looked like wolf bites. He named the rash Discoid (Round Shape) Lupus Erythematosus (DLE).

4. INTRODUCTION In 1885, Sir William Osler recognized that many people with lupus had a disease involving not only the skin but many other organs or systems. He named the disease Systemic Lupus Erythematosus (SLE).

5. EPIDEMIOLOGY Overall population, the global SLE incidence and newly diagnosed population were estimated to be 5.14 (1.4 to 15.13) per 100000 person-years and 0.40 million annually, respectively.

6. TYPES OF LUPUS

7. Systemic Lupus Erythematosus (SLE) One that most people refer to when they say “lupus”. The symptoms of SLE may be mild or serious. Although SLE usually first affects people between the ages of 15 and 45, it can occur in childhood or later in life as well.

8. Discoid Lupus Erythematosus (DLE) A chronic skin disorder in which a red, raised rash appears on the face, scalp, or elsewhere. The raised areas may become thick and scaly and may cause scarring. The rash may last for days or years and may recur. A small percentage of people with discoid lupus have or develop SLE later.

9. Neonatal Lupus A rare disorder that can occur in newborn babies . Researcher suspect that neonatal lupus is caused by auto-antibodies in the mother’s blood called anti-Ro (SSA) and anti-La (SSB). Auto-antibodies (“ aut o” means “self”) are blood proteins that act against the body’s own parts. At birth , the babies have a skin rash, liver problems, and low blood counts. These symptoms gradually go away over several months, although in rare cases.

10. CAUSES Unknown , but it is likely that a combination of genetic , environmental , and, possibly, hormonal factors work together to cause SLE. This occurs through the production of “ auto-antibodies ” that attack a person’s own cells thus contributing to the inflammation of various parts of the body , and may cause damage to organs and tissues . The most common type of auto-antibody that develops in people with SLE is called an antinuclear antibody (ANA) because it reacts with parts of the cell’s nucleus. The fact that SLE can run in families indicates that its development has a genetic basis ; however, no specific “lupus gene” has been identified yet.

11. CLINICAL FEATURES C A R D I A C Endocarditis Myocarditis Pericarditis General Body Changes Fatigue Fever Weight loss G A S T R O I N T E S T I N A L Abdominal pain Nausea & vomiting

12. CLINICAL FEATURES D E R M A T O L O G I C A L Alopecia Butterfly rash Mucous membrane lesion Photosensitivity Purpura Raynaud’s phenomenon Urticaria Vasculitis

13. CLINICAL FEATURES H E M A T O L O G I C Anemia Leukopenia Thrombocytopenia M U S C U L O S K E L E T A L Arthralgia Arthritis Myositis RESPIRATORY Pleurisy Pulmonary hypertension

14. CLINICAL FEATURES N E U R O P S Y C H I A T R I C Cranial neuropathies Organic brain syndrome Peripheral neuropathies Psychosis Seizures Transverse myelitis M U S C U L O S K E L E T A L Casts Hematuria Nephrotic syndrome Proteinuria

15. DIAGNOSIS Diagnosis of systemic lupus erythematosus (SLE) is based on clinical symptoms & lab findings American College of Rheumatology criteria for the diagnosis of definite lupus in children ≥4 criteria on the list either at the present time or at some time in the past, there is a strong chance that Client have lupus.

16. DIAGNOSIS 11 common criteria, or measures that was developed by the American College of Rheumatology (ACR) Malar rash – a rash over the cheeks & nose, often in the shape of a butterfly Discoid rash – a rash that appears red, raised, disk-shaped patches. Photosensitivity – a reaction to sun or light that causes a skin rash to appear or Oral Ulcers – sores appearing in the mouth Arthritis – joint pain & swelling of 2 or more joints in which the bones around the joints do not become destroyed. Serositis – inflammation of the lining around the lungs (pleuritis) or (pericarditis) Kidney disorder – persistent protein or cellular casts in the urine. Neurological disorder – seizures or psychosis Blood disorder – anemia, leukopenia, lymphopenia, or thrombocytopenia Immunologic disorder – anti-DNA or anti- Sm or positive antiphospholipid antibodies Abnormal antinuclear antibody (ANA)

17. DIAGNOSIS Diagnosis of systemic lupus erythematosus (SLE) is based on clinical symptoms & lab findings Diagnosis based on the Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for systemic lupus erythematosus (SLE) ≥4 criteria (at least 1 clinical & 1 immunologic criteria) or Biopsy-proven lupus nephritis with positive   antinuclear antibody (ANA ) or Anti-double stranded deoxyribonucleic acid ( dsDNA ) Symptom/finding need not be present all at the same time

18. DIAGNOSIS I M M U N O L O G I C A L C R I T E R I A: Antinuclear antibodies (ANA) level above laboratory reference range Anti-double stranded deoxyribonucleic acid ( dsDNA ) antibody level above laboratory reference range [ or >2-fold the reference range if tested by enzyme-linked immunosorbent assay (ELISA)] Anti-Smith (Anti- Sm ): presence of antibody to Smith ( Sm ) nuclear antigen

19. DIAGNOSIS I M M U N O L O G I C A L C R I T E R I A: Antiphospholipid antibody positivity, as determined by: Positive test for lupus anticoagulant False-positive test result for rapid plasma reagin Medium- or high-titer anticardiolipin antibody level  [Immunoglobulin A (IgA), immunoglobulin G ( IgG ) or immunoglobulin M ( IgM )] Positive test result for anti-B2-glycoprotein I [Immunoglobulin A (IgA), immunoglobulin G ( IgG ) or immunoglobulin M ( IgM )] Low complement (C3, C4, or CH50) Direct Coombs’ test (in the absence of hemolytic anemia)

20. Diagnostic Measures History-taking Physical exam Lab tests Complete blood count (CBC) Creatinine measurement Urinalysis

21. Diagnostic Measures Decrease in serum complement levels Increase in anti-double stranded deoxyribonucleic acid (dsDNA) Increase in erythrocyte sedimentation rate (ESR) Decrease in hemoglobin level, leukocyte or platelet counts Increase in creatine phosphokinase (CPK) levels Appearance of microscopic hematuria or proteinuria

22. G O A L S O F T H E R A P Y : Control disease manifestation Allow child to have a good quality of life without major exacerbations Prevent serious organ damage that adversely affects function or lifespan Prevent adverse effects of the drugs used PHARMACOTHERAPY Corticosteroids Immunosuppressants NSAIDs Sunscreen Management

23. C O R T I C O S T E R O I D S Oral corticosteroids Patients w/ mild SLE do not normally require use of systemic corticosteroids but there are patients who has low quality of life if not given low-dose with maintenance therapy High-dose for severe organ systems’ manifestations especially CNS, renal & hematologic manifestations Decreases inflammation by suppression of the immune system Topical corticosteroids Helpful for discoid lesions especially on the scalp Use a less potent steroid on the face because it is more prone to atrophy Parenteral corticosteroids Pulse therapy with IV corticosteroids in combination with immunosuppressive therapy is recommended for Class III and IV SLE patients with confirmed glomerulonephritis Pharmacological Management

24. H Y D R O X Y C H L O R O Q U I N E Used for skin & joint manifestations Also used for preventing flares & other constitutional symptoms Inhibits chemotaxis of eosinophils & locomotion of neutrophils & impairs complement-dependent antigen-antibody reactions Recommended as background treatment for Class III and IV SLE patients with nephritis . Pharmacological Management

25. I M M U N O S U P P R E S S A N T S These agents act as immunosuppressive, cytotoxic & anti-inflammatory agents In the treatment of severe CNS & severe glomerulonephritis, thrombocytopenia & hemolytic anemia, high dose glucocorticoids & immunosuppressants are used Azathioprine Belimumab Cyclophosphamide IV Immune Globulin (IVIg) Methotraxate Pharmacological Management

26. N S A I D S These drugs provide symptomatic relief of fever, arthritis & mild serositis Inhibit inflammatory reactions & pain by decreasing prostaglandin synthesis SLE patients have a high incidence of NSAID-induced hepatotoxicity. S U N S C R E E N Patients with SLE should apply sunscreen with at least an SPF of 15 to prevent dermal or systemic disease flares upon exposure to ultraviolet light Pharmacological Management

27. Complications Some degree of long term and often permanent organ dysfunction from either SLE or its treatment has been found in 88% of patients. Hypertension Growth retardation Chronic pulmonary impairment Ocular abnormalities Permanent renal damage Neuropsychiatric symptoms Musculoskeletal damage Gonadal impairment