Differential Diagnosis

Systemic lupus erythematosus (SLE): when active, usually a homogenous pattern on ANA or less commonly speckled, rim, or nucleolar when present in high enough titer to be clinically significant

Sjögren syndrome: common ANA pattern is speckled; less commonly homogenous

Scleroderma or systemic sclerosis: ANA pattern is most commonly speckled, followed by centromeric and less commonly nucleolar


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Mixed connective tissue disease: ANA pattern is almost always speckled

Rheumatoid arthritis (RA): when ANA positive (about 25-40% of cases), a speckled ANA pattern most commonly

Don't Miss – Dangerous Situations

These are all chronic diseases, and, if there is a life-threatening complication, the complication could be serious.

Commonly Encountered Situations

SLE

  • Common autoimmune disorder

  • Much more common in women than men (female:male ratio approximately 9:1)

Suggested Additional Lab Testing

For follow-up from a positive ANA test:

  • For SLE: anti Ds DNA, anti-SS-A (Ro), anti-SS-B (La), anti-U1 RNP, anti-Sm, and follow-up with serial anti-DS DNA

  • For Sjögren syndrome: anti-SS-A (Ro), anti-SS-B (La)

  • For polymyositis and adult dermatomyositis: anti-Jo-1

  • For mixed connected tissue disease: test for anti-U1 RNP

  • For scleroderma: if there is a fine or grainy nuclear pattern, test anti-Scl-70 (topoisomerase I); if there is a centromeric pattern, no additional tests are recommended.

In addition to the follow-up with serologic testing, patients with Sjögren syndrome can be tested for dry eyes and dry mouth by a variety of assays.

Patients with possible inflammatory muscle disease may benefit from creatine kinase testing and/or a muscle biopsy. Evaluation for RA: rheumatoid factor test (found in approximately 80% of patients with RA); x-rays, particularly of hand and wrist for joint disease; C-reactive protein test as an indicator of inflammation with or without SED rate to assess level of inflammation.