In July, a 24-year-old single woman visits her family physician because of a flu-like illness that began two weeks ago with fever and muscle and joint pains. The symptoms appeared two days after getting sunburned at Jones Beach on Long Island.

She also reports having episodes of fatigue and muscle pains lasting two to three days over the past six months, which she attributes to emotional stress on her job as a paralegal. She has treated these episodes by taking up to eight regular aspirin daily with only temporary relief.

She has no history of a tick bite and has had no major illnesses. She does not smoke. She drinks alcoholic beverages regularly, but does not get drunk. Family history is significant for a maternal aunt who had her spleen removed for an “immune bleeding problem.”

A. RS³PE syndrome
B. Parvovirus B19
C. Systemic lupus erythematosus
D. Rheumatoid arthritis
E. Hepatitis B
F. Fibromyalgia syndrome
G. Polymyalgia rheumatica
H. HIV-related arthritis
I. Lyme disease

On further questioning, she reports that her hand joints are painful with movement and her knuckles seem swollen. Her back has not been painful or stiff. For the past two years she has noticed that her fingers become pale and then bluish and painful when she spends a lot of time swimming or they are exposed to cold temperatures.  She also reports multiple mouth sores in the past four to six weeks. She suffered from a prolonged episode of “growing pains” when she was 12 years old, and there was some concern that she might have had rheumatic fever, but this was not confirmed.

She denies having any of the following symptoms: muscle weakness; headache, confusion, memory loss, or visual changes; hair loss, dry eyes, dry mouth, or hearing loss; sinusitis or ocular inflammation; cough, chest pain, hemoptysis, or pleurisy; heart murmurs, dyspnea on exertion, or palpitations. A PPD skin test one year ago was negative.

She has had no anorexia, weight loss, nausea, vomiting, diarrhea, heartburn, or abdominal pain; stools are regular, brown, and have no signs of bleeding or mucus. Her last menstrual period was three weeks ago and was normal. She has regular sexual contact with her boyfriend. She has had two to three urinary tract infections over the past two years, but no history of venereal disease. She has not noticed bruising, easy bleeding, or swollen lymph nodes. She has no history of pregnancy, phlebitis or “blood clots,” seizures, psychosis, hallucinations, cognitive changes, incoordination, mood disorder, or any major psychiatric disease. She has no history of intravenous drug use, and on careful questioning, she has no known risk factors for HIV infection.

Physical examination reveals an ill-appearing young woman. Her temperature is 99.8^oF; pulse 100, and BP 110/65. She is 5’4" and weighs 110 lbs. A mild malar blush is evident. Her throat is injected, with a shallow palatal ulcer. The thyroid is not enlarged or tender. There are many shotty, mobile, tender anterior and posterior cervical lymph nodes. Lung sounds are clear, and heart sounds S1 and S2 are normal, but there is a grade 2/6 systolic murmur at the apex without radiation. Her abdomen is mildly tender in the upper quadrants, but the liver edge is not palpable, but the spleen tip is felt on full inspiration.

She has mild but definite joint swelling and tenderness of both wrists, PIPs, MCPs, and knees. No objective muscle weakness is evident, but her arm and leg movements are guarded because of pain. She does not exhibit painful tender points in areas other than the affected joints.

On neurologic examination, she is alert and oriented. Cranial nerves are intact, and her gait and coordination are normal. Deep tendon reflexes are 2+ and bilaterally symmetrical. Cutaneous sensation is intact, and there are no pathological reflexes.

Blood and urine samples are taken for analysis, and a throat swab is obtained for strep culture. She is started on ampicillin 250 mg qid.

A. RS³PE syndrome
B. Parvovirus B19
C. Systemic lupus erythematosus
D. Rheumatoid arthritis
E. Hepatitis B
F. Fibromyalgia syndrome
G. Polymyalgia rheumatica
H. HIV-related arthritis
I. Lyme disease

Four days later, she calls because her symptoms have not improved, and now she has developed a nonpruitic facial and truncal rash. The physician advises her to discontinue the ampicillin and come to the office the following day. The patient continues the aspirin.

The test results obtained on the initial visit are:

Results of other tests are normal or negative. Urinalysis, BUN/creatinine, electrolytes, and liver enzymes are normal, as is the ELISA for Lyme disease. Both the throat culture and ASO titer are negative for strep, and the Monospot test, HIV antibody, and hepatitis B and C serology are all negative.

A. RS³PE syndrome
B. Parvovirus B19
C. Systemic lupus erythematosus
D. Rheumatoid arthritis
E. Hepatitis B
F. Fibromyalgia syndrome
G. Polymyalgia rheumatica
H. HIV-related arthritis
I. Lyme disease

On physical examination, her temperature is now 101^oF. She has raised, erythematosus, blanching, coin-sized blotches with no central clearing on her cheeks, forehead, ears, arms, back, and chest.  Blotchy red spots have appeared on the palmar aspects of her hands and fingers.

Her palate is red and raw, with scattered vesicular lesions but no pustules.  No pleural or pericardial rubs are heard. Otherwise, physical examination is unchanged.

A chest x-ray demonstrates some blunting of the left costophrenic angle. Joints appear normal on radiographs. Additional blood and urine testing is done.

A. ESR: 45 mm/hr
B. CBC: Hgb/Hct 11.0 gm/dl/30%; WBC 3,000/mm³; platelets 165,000/mm³
C. Urinalysis: Normal
D. Renal function tests: BUN 12 mg/dl, creatinine 0.8 mg/dl
E. Rheumatoid factor: Negative
F. ANA: 1:2,560 in a speckled pattern
G. VDRL: Negative
H. Joint radiographs: Normal

Question 5: Which two tests would you do next?

A. Skin biopsy
B. Kidney biopsy
C. Echocardiogram
D. Anti-dsDNA antibodies
E. Serum complement levels
F. Lymph node biopsy
G. Liver/spleen scan
H. Pulmonary function tests

After the patient failed a trial of NSAID therapy, prednisone 20 mg daily and hydroxychloroquine 400 mg daily is prescribed. Her clinical symptoms and laboratory values improve, and after four weeks she is able to taper off the prednisone.

She is referred to occupational therapy for hand evaluation, potential splinting and joint protection, and is counseled regarding prevention of Raynaud’s phenomenon.

After working this case, the student should be able t

1. Describe at least seven clinical manifestations of lupus.
   
   
2. List three laboratory tests or sets of tests important in the initial evaluation of possible lupus.
   
   
3. List at least six causes of acute polyarthritis with fever.
   
   
4. Recommend appropriate work-up of the patient with clinical findings suggesting systemic vasculitis.

Boumpas DT, Fessler BJ, Austin III HA, et al. Systemic lupus erythematosus: emerging concepts. Parts I and II. Ann Intern Med 1995;122:940-50 and 123:42-53.

Egner W. The use of laboratory tests in the diagnosis of SLE. J Clin Pathol 2000;53(6):424-32.

Hahn BH. Antibodies to DNA. N Engl J Med 1998; 338(19):1359-68.

Tan EM. Antinuclear antibodies: diagnostic markers for autoimmune diseases and probes for cell biology. Adv Immunol 1989;44:93-151.

Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982;25:1271-7.

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