Systemic lupus erythematous (SLE) is a chronic and systemic autoimmune disease in which tissue binding autoantibodies and immune complexes attach to any tissue or organ throughout the body causing chronic inflammation. This autoimmunity is caused by a combination of genetic susceptibility to an unregulated immune system and an environmental stressor such as sunlight or infection.  90% of SLE patients are women of child bearing age, with 20-120 US women per 100,000 affected by the disease. SLE is most common in African-American women and least common in white men. However, the disease can occur in people of all ages, sexes and ethnic groups¹.

A 46-year-old Caucasian woman presents with a 6 month history of generalized weakness, pain in her elbows and knees, and 40 lbs. weight loss over 4 months. The patient is obese and, due to financial issues, has unmanaged hypertension (high blood pressure). She has been treated for leukopenia (decreased number of white blood cells) on more than two occasions. She recently noted that her urine had a 'foamy' appearance for the past month.

Vitals

• BP: 144/88
• HR: 88 bpm
• Temp: 98.6°F
• RR: 24 breaths/min
• Ox/Sat: SpO₂ of 92%
• BMI: 31

Lab Tests

• CBC: Anemia (Hb 11.5g/dL), leukopenia (~3,500/mm³ of whole blood)
• BUN: Elevated (25mg/dL)
• Creatinine: Elevated (1.6mg/dL)
• Urinalysis: Trace proteinuria (15mg/dL)

Systemic Lupus Activity Measure

• Revised Systemic Lupus Activity Measure (SLAM-R): 20:81 (tests for 23 clinical manifestations of SLE and is scored on a 0-81 range with a score above 7 being considered significant).

Blood Antibody Test 

• High anti-nuclear antibody (ANA), anti-dsDNA antibody, and anti-Smith antibody titers.

Physical Exam

• Dullness to percussion plus an absence of breath sounds on left lung.
• Generalized weakness with limited, painful range of motion in the elbows and knees.
• Macular rash and flushing of cheeks, i.e. butterfly rash (see below).     

Butterfly rash

Treatment

• Physical therapy to alleviate joint pain while increasing range of motion.
• Application of moist heat ≤ 5 minutes BID (two times a day) for joint pain.
• Limit UV exposure to prevent worsening of macular rash.
• Recommend monitoring kidney function with primary physician.
• Strategies for stress reduction were discussed. 

The patient visited the hospital and complained of 'all over' body weakness for the past 6 months. She was concerned about her 40 lb weight loss of the past 4 months and her trouble breathing for the past 2 weeks. She works as a computer programmer and took a 2 week leave from her job due to her inability to work for long periods of time.  Because of constant fatigue and joint pain, particularly elbows and knees, she has been unable to do yard work and some routine activities like house cleaning. The patient tried to limit her physical exertion in an attempt to alleviate her symptoms, but it only slightly helped.  Since she lives alone and is not married financial constraints are a big concern. Due to this, her hypertension has gone unmanaged and she is no longer able to afford the previously prescribed Lozol (indapamide).  She is worried that her chronic symptoms will hinder her ability to continue working or identify future employment that does not exacerbate her current condition. Also, she desires to perform routine activities with less pain and fatigue.

The subtypes of SLE differ in the tissue or organ system(s) affected. When affecting the skin, the cutaneous presentation of lupus (cutaneous lupus erythematosus [CLE]) may either be SLE-associated or independent². The patient presented with the classic “butterfly” rash on the face, which is suggestive of CLE. A patient is considered to have mild-SLE if their body systems are functioning normally due to mild disease presentation or responsive drug treatment. In contrast, SLE is classified as uncontrolled when the patient presents with inflammation such as pleurisy or arthritis. Uncontrolled SLE does not respond to treatment, e.g. NSAIDS and topical corticosteroids, causing additional manifestations of the disease to present². One noticeable manifestation is the decreased kidney function noted by the increased BUN, creatinine, and protein in her urine. The patient's state of health is indicative of uncontrolled SLE, as chronic arthritis, rash, and muscle weakness were noted. The onset of these symptoms is due to the immunopathology of lupus. Lupus, an autoimmune disease, involves activation of the innate immunity by DNA in immune complexes, viral DNA/RNA, and RNA protein self-antigens. Furthermore, empirical evidence suggests that an abnormal adaptive immunity pathway contributes to the disease's progression due to ineffective regulatory CD4 T cell, CD8 T cell, B cell, and suppressor cell responses². The reduced clearance of apoptotic cells and immune complexes is contributed to by decreased phagocytic function from overactive immune cells and the chronic release of inflammatory mediators (IFN, TNF, IL, etc.). Collectively, this causes the classic tissue damage that is seen in CLE, i.e. the ‘butterfly’ rash. Lastly, the continuous production of auto-antibodies and immune complexes are responsible for the joint pain, which is usually an early indicator of lupus. The probability of lupus was confirmed by the presence of the elevated anti-dsDNA, anti-Smith, and ANA antibodies². Based on the lab results, in addition the presence of multiple clinical manifestations via the semiquantiative SLAM-R, one could conclude that a diagnosis of lupus is appropriate.