Patients with connective tissue diseases and vasculitides can present to the ED with vague or seemingly innocuous complaints but may actually have a life threatening condition.

Key conditions to assess for include:

1. Septic arthritis in the rheumatoid arthritis (RA) patient

  • Can be difficult to diagnose, especially in the earlier stages of infection, where an acutely swollen joint may be presumed to be due to the patient’s underlying RA
  • Delay in diagnosis of septic arthritis can lead to irreversible joint damage and has a mortality rate of 20-33%
  • Patients with longstanding RA and those who are receiving immunosuppressive therapy are at higher risk for septic arthritis
  • Suspected joint should be tapped with assessment for cell count and differential, glucose, gram stain and culture, and crystal analysis

2. Atlantoaxial subluxation in the RA or ankylosing spondylitis (AS) patient

  • Tend to occur in patients with erosive long-standing RA or AS
  • Forward dislocation is most common (43% of long-standing RA pts)
  • concerning signs for an unstable cervical spine include new occipital pain, decreased sensation in extremities
  • CT and/or MRI recommended to assess for subluxation

3. Cricoarytenoid arthritis in the RA patient

  • can be asymptomatic but patients may also present with hoarseness, foreign body sensation, inspiratory stridor, or in severe cases, with acute respiratory distress/failure
  • Laryngoscopy showing edema and arytenoid cartilage asymmetry or CT can diagnose the condition

4. Scleroderma renal crisis

  • Associated with 20% mortality
  • More likely to develop in patients with early systemic sclerosis
  • Classic signs/symptoms include headache, severe hypertension, elevated creatinine, and evidence of microangiopathic anemia with thrombocytopenia

5. Alveolar hemorrhage in the systemic lupus erythematosus (SLE) patient

  • Rare but significant complication of SLE with mortality of up to 70-90%
  • Patients typically have dyspnea with pulmonary infiltrates, fever and less commonly hemoptysis
  • Early treatment with high dose steroids +/- cyclophosphamide is recommended

6. Transverse myelitis in the SLE patient

  • Occurs in approximately 2% of SLE patients
  • If not recognized and treated early, can lead to irreversible paraplegia
  • Patients typically present with weakness, paresthesias, or lower extremity pain, often associated with back pain and bladder dysfunction
  • If TM is suspected, an MRI should be obtained to support the diagnosis
  • Treatment with steroids, cyclophosphamide, and plasmapheresis is recommended

7. Catastrophic antiphospholipid syndrome (CAPS)

  • Patients with SLE or primary antiphospholipid syndrome are at risk for developing CAPS (often in setting of infection, trauma, surgery, or cancer)
  • Presents most commonly present with renal impairment, pulmonary disease (PE or ARDS), cerebral findings (infarcts, seizures), thrombocytopenia
  • Other thrombotic complications include MI, skin necrosis
  • Treatment involves IV heparin and high dose steroids

8. Cardiac tamponade

  • Patients with widespread active SLE can rarely present with cardiac tamponade
  • Symptoms typically include dyspnea, joint pains, fatigue, and chest pain

9. Hypokalemic paralysis in the Sjogren’s syndrome (SjS) patient

  • Patients with SjS can develop distal renal tubular acidosis which can lead to significant hypokalemia and potentially, paralysis
  • Treatment involves potassium replacement and supportive care

10. Giant cell arteritis

  • Temporal arteritis, causing visual impairment is a significant complication in patients with GCA. Steroids are indicated in any patient with symptoms/signs suggestive of temporal arteritis to prevent permanent visual loss
  • GCA can also cause vertebro-basilar insufficiency, which can present with gait impairment, dizziness or vertigo, nausea/vomiting
  • If untreated, vertebro-basilar insufficiency in patients with GCA can progress to bilateral vertebral artery occlusion, with a mortality of approx. 75%
  • MRI or MRA may differentiate GCA involvement of vertebral arteries from an atherosclerotic process
  • Aortitis can also develop in patients with GCA and can cause aortic aneurysm and rupture, dissection, and aortic valve disease

 

Slobodin G, Hussein A, Rozenbaum M, Rosner I. The emergency room in systemic rheumatic diseases. Emerg Med J 2006; 23: 667-671.