A 30 yo F with hx of migraine headaches presents with bilateral leg swelling and shortness of breath. Also complains of hair loss and intermittent hand, knee, and elbow pain.

Questions to Ask

  • Onset of presenting symptoms?
  • Any other symptoms?
  • Does the patient have hematuria?
  • Any drug use? Supplements? Over-the-counter pain medications such as NSAIDs?
  • Family history of autoimmune disease?

History & Physical Exam

  • Rash and joint pain started last week during a family trip to Florida.
  • Hand pain is in her wrists and MCPs.
    • No swelling, no morning stiffness.
    • Pain is mild but noticeable.
  • She has never had these symptoms before.
  • No alopecia, oral or nasal ulcers, chest pain, shortness of breath, or abdominal pain.
  • Physical exam:
    • Rash on the cheeks and nose, sparing the nasolabial folds.
    • Wrists and MCPs are tender but not swollen.

Diagnostic Workup

  • Recommended initial labs: CBC with diff, CMP, ANA by IFA, anti-dsDNA, anti-Smith, SSA, SSB, complement levels, ESR, CRP, urinalysis, urine protein/creatinine ratio
  • Recommended imaging: echocardiogram

Referrals

  • Refer to rheumatology
  • Refer to dermatology

Treatment & Management

  • Given high pretest probability of lupus, and in the setting of a malar rash, can initiate treatment for acute flare of lupus
  • Can start hydroxychloroquine (weight-based dosing not to exceed 5 mg/kg)
  • Can also start topical steroid or systemic steroids to treat acute flare (e.g. prednisone 15-20 mg per day with a taper over ~4 weeks) for symptom relief
  • Monitoring blood sugars, blood pressure, volume overload/heart failure symptoms

A 25 yo F with no significant PMH presents with a rash on her face in a malar distribution. She also complains of pain in her hands.

Questions to Ask

  • Inflammatory?
  • Duration of symptoms?
  • Onset of rash?
  • Joint involvement? Bilateral?
  • Any other symptoms?
  • Triggers (e.g., recent sun exposure)?

History

  • She developed leg swelling 2 weeks ago
  • Also noticed that her face is swollen in the morning, especially around her eyes
  • Has been getting progressively short of breath since then
  • First noticed a bald spot on her scalp 2 months ago
  • Has had intermittent arthralgias for 6 months
  • Has noticed her urine has become foamy and slightly pink

Diagnostic Workup

  • Recommended initial labs: CBC with diff, CMP, ANA by IFA, anti-dsDNA, anti-Smith, SSA, SSB, complement levels, ESR, CRP, RF, CCP (due to joint involvement)
  • Recommended imaging: hand X-rays – 3 views
  • If urine studies are consistent with glomerulonephritis (GN), must rule out infection and recommend renal biopsy to evaluate for lupus nephritis

Referrals

  • Given high pretest probability of lupus nephritis, workup should be expedited and patient should be urgently evaluated by a rheumatologist and a nephrologist.
  • If cannot be seen within a few days, refer for hospital admission.

Treatment & Management

  • Start hydroxychloroquine (weight-based dosing not to exceed 5 mg/kg)
  • High-dose steroids: 0.5-1 mg/kg for ~3 days followed by long taper
  • Add immunosuppressive therapy (mycophenolate or cyclophosphamide)
  • Patient must be monitored closely to ensure response to therapy and to evaluate for possible relapse