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Systemic Lupus Erythematosus (SLE)

Contributors: Elizabeth Brooks, MD and Michelene Hearth-Holmes, MD

CASE 1:

A 21 year old college student presents with a two month history of multiple joint pains that are worse in the morning. She has noticed a faint red rash on her face for the last month that is photosensitive. She is very tired and finds it difficult to concentrate during classes. She denies any fevers, abdominal pains, diarrhea, constipation or chest pains.

Her physical exam reveals a red malar rash that spares the nasolabial folds. On lung exam there are decreased breath sounds at the bases. There are no murmurs. Musculoskeletal exam reveals mild synovitis of the metacarpophalangeal (MCP) joints and proximal interphalangeal (PIP) joints bilaterally. There is diffuse cervical adenopathy. The remainder of the exam is normal.

Laboratory exam reveals that the white count is 3.2 with normal Hemoglobin and platelets. Rheumatoid factor (RF), anti-CCP antibodies and thyroid studies were normal. The urinalysis reveals two plus proteinuria, without casts or red cells. The urine protein/creatinine ratio was 0.3 (300 mg/24hrs).  An anti-nuclear antibody (ANA) was 1:640 (by immune fluorescence) and the anti- dsDNA antibody was positive. Further testing revealed a positive anti- Smith (Sm) antibody. A CXR revealed small bilateral pleural effusions.

A diagnosis of systemic lupus erythematosus (SLE) was made. What clinical and laboratory results were used to confirm the diagnosis of SLE?

  • This patient with SLE presented with arthritis, malar rash, decreased breath sounds and lymphadenopathy. Over 50% of SLE patients will present with adenopathy that is small and symmetrical.  Fatigue is common in over 90% of patients. The concern with this presentation is that she may have an underlying infection or malignancy. This patient had an extensive infectious disease work up as well as a lymph node biopsy which was negative. The arthritis in these patients is usually of the small joints of the hands (MCPs and PIPs) and is nonerosive.
  • Her positive lab tests help confirm the diagnosis of SLE. The ANA is positive as well as the anti-ds DNA antibody and the anti-Sm antibody. The last two are specific for the diagnosis of SLE. She has a low white count, proteinuria, and bilateral pleural effusions.  All of these can be seen in SLE.
  • American College of Rheumatology (ACR) criteria for the diagnosis of SLE states that a patient must have four of 11 criteria. This criteria is primarily for standardization of research protocols, but it is helpful in identifying patients. This patient had 7 of the 11 criteria (positive ANA, arthritis, malar rash, photosensitivity, serositis, leukopenia, proteinuria, positive anti-ds DNA antibody  and a positive anti-Sm antibody)
  • It is necessary to closely watch this patient's blood pressure and urinalysis over the next 12 months. She is at risk for lupus nephritis. An increase in proteinuria or increased activity of the urinary sediment may require that this patient have a kidney biopsy so that treatment can be stratified according to biopsy results.

CASE 2:

A 24-year old woman with a past medical history of SLE for four years presents with two weeks of fatigue and joint aches.  Her prior lupus manifestations include: type IV lupus nephritis, positive ANA, positive anti-dsDNA antibody and anemia. She has been stable for two years on mycophenolate mofetil and hydroxychloroquine. She has not required prednisone for 12 months.

She denied any fevers, cough or sore throat. She started taking acetaminophen and had some relief. This week she developed chest pain that was not relieved with acetaminophen. She came into the clinic with shortness of breath and moderate chest pain. She is unable to sleep and is tired all day.

On physical examination, her blood pressure is mildly elevated and she was afebrile. She has decreased breath sounds diffusely. Her cardiovascular exam was normal. She has pain in the small joints of her hands and feet without synovitis. There is no edema in the lower extremities and pulses are normal.

A CXR showed small bilateral pleural effusions with mild cardiomegaly. A transthoracic echocardiogram revealed a moderate pericardial effusion. Lower extremity dopplers and a Chest CT scan were done and were negative for DVT and PE, respectively. Laboratory results revealed a normal CBC, CMP and urinalysis.

Forty mg of prednisone was started for a flare of her SLE, with moderate pericardial and pleural effusions. She had immediate relief, but returned in one week with burning pain on the right side of her chest. The pain was intense and on physical exam you see multiple blisters on her right anterior chest with radiation to her axilla. You diagnose herpes zoster and admit her to the hospital for IV acyclovir.

What are some of the other complications or infections that SLE patients may encounter due to their disease and/or the use of immunosuppressive medications?

This patient has had SLE since age 20. She has been treated for Type IV lupus nephritis with good results. Her creatinine and urinalysis are normal and she has been stable for one year without prednisone. She has developed a further manifestation of SLE with serositis manifesting as pleural effusions and a moderate pericardial effusion. NSAIDS and glucocorticoids are excellent treatment for these symptoms. This patient could not take NSAID's due to her underlying kidney disease so prednisone was restarted. Patients taking prednisone and other immunosuppressives are at risk for opportunistic infections (bacterial, viral and fungal) that can be serious and sometimes life threatening. This patient developed herpes zoster and did well on acyclovir.  It is important to be aware of the need to quickly assess for acute and chronic infections in any patient with SLE. It is more likely that the patient had an infection rather than an exacerbation of her primary disease of SLE following the use of high dose prednisone for her serositis. Other complications of glucocorticoids use are weight gain, diabetes, acne, mood instability, osteoporosis, osteonecrosis and an increased risk of cardiovascular disease.

Patient Care:

  1. Be able to list the diagnostic criteria for systemic lupus erythematosus
  2. Obtain the appropriate history to determine if a patient might have lupus
  3. Recognize the clinical manifestations of systemic lupus erythematosus
  4. Recognize when ANA testing should be utilized, and determine the appropriate serologies to diagnosis lupus
  5. Determine who should be referred to a rheumatologist for evaluation of a positive ANA
  6. Identify the most common serious complications of systemic lupus
  7. Recognize serious infection in a patient with systemic lupus on immunosuppressant therapy
  8. Identify the most common causes of early mortality in patients with systemic lupus
  9. Recognize the high incidence of cardiovascular disease in patients with SLE
  10. Identify the antiphospholipid syndrome and tests used to screen for antiphospholipid antibody and its close association with lupus.
  11. Recognize the need for ongoing monitoring of patients with lupus
  12. Identify treatment strategies for the myriad complications of lupus
  13. Recognize pregnancy implications in women with lupus

Medical Knowledge:

  1. Apply knowledge of lupus manifestations to discuss issues important to a primary care provider caring for patients with lupus (includes bone health, reproductive issues and appropriate contraception, cardiovascular disease screening)
  2. Interpret results of ANA and ENA testing
  3. Interpret the results of anti-phospholipid antibody testing
  4. Determine which population subgroups are at greatest risk for development of lupus
  5. Identify the causes of hypocomplementemia in patients with systemic lupus
  6. Identify the drugs used most often to treat lupus and the potential toxicities
  7. State the limitations of the lupus criteria
  8. Describe the different types of lupus nephritis
  9. Recognize the pulmonary, cardiac, renal, gastrointestinal, hematologic, neurologic, musculoskeletal and cutaneous manifestations of lupus

Practice-based Learning and Improvement:

  1. Set goals for learning about lupus, its manifestations and treatment
  2. Demonstrate the ability to review and interpret the literature relevant to the care of patients with lupus
  3. Recognize the importance of learning from mistakes to improve patient care in the future.

Interpersonal and Communication Skills:

  1. Discuss the results of ANA testing with a patient
  2. Discuss the results of anti-phospholipid antibody testing with a patient and the potential implications of a positive test
  3. Discuss the diagnosis of lupus with a patient in a way that incorporates a patient's perspective.
  4. Discuss the need for appropriate follow up and monitoring with a patient
  5. Explain the different treatment options based on disease manifestations
  6. Discuss pregnancy risks associated with active lupus
  7. Discuss the long term risks of lupus and implications for early atherosclerotic disease

Professionalism:

  1. Recognize the importance of patient confidentiality and privacy, and demonstrate integrity and honesty in discussing patient care issues and management with the patient and family.
  2. Understand the need to provide supportive care
  3. Encourage patients to ask questions regarding their disease and empower them to be participants in their care
  4. Provide adequate time and accessibility to address patient concerns
  5. Demonstrate an understanding of informed consent with regard to therapeutic options in the treatment of lupus

Systems-based Practice:

  1. Create a team approach to manage patients with lupus engaging physicians from multiple disciplines
  2. Identify barriers to access and delivery of care to patients with complex, multi-system diseases
  3. Understand the resources available to help with delivery of care and patient support
  4. Identify the ways in which one can be a patient advocate including obtaining prior authorization for medications, treatments and diagnostic tests
  5. Demonstrate an awareness of the economic implications of a diagnosis of lupus for both the patient as well as the potential burden on the healthcare system

Keywords: Systemic lupus erythematosus, Lupus, Antiphospholipid antibody syndrome, Anti-Nuclear Antibody, ANA, Lupus Nephritis

References