Sjögren Syndrome (Sicca Syndrome, Mikulicz Disease)

Figure 3.35 A: Morphea (Courtesy of Dr. Paul Getz) B: Morphea (Courtesy of Dr. Sophie M. Worobec) C: Morphea

• Chronic autoimmune disorder affecting exocrine gland function with xerophthalmia (dry eyes or keratoconjunctivitis sicca), xerostomia (dry mouth) and arthritis
• May be primary disorder or associated with other autoimmune diseases such as SLE
• Antibodies: anti-α-fodrin (70%), anti-Ro and anti-La
• Presents with xerosis of the mucous membranes (mouth, eyes, vagina) and skin (pruritus), fatigue and arthritis; xerophthalmia with foreign body sensation, positive Schirmer test (detects ↓ lacrimal gland secretion: paper wick placed over lower eyelid for 5 min, abnormal with typically <5 mm of moistening) and subsequent corneal ulceration and keratitis; ± vasculitis, renal involvement, parotid gland enlargement, peripheral neuropathy, lymphadenopathy with ↑ risk of lymphoma
• Labs: ↑ ESR, + rheumatoid factor, + anti-fodrin, + anti-Ro, + anti-La; leukopenia
• Histology: dense lymphocytic infiltrate surrounding minor salivary glands (need presence of two or more aggregates for diagnosis)
• Treatment: mainly supportive; artifical tears, cyclosporine eye drops, methylcellulose drops (artificial saliva), sugarless water, etc.; immunosuppressants for patients with vasculitis or internal organ involvement
• Associated with HLA-B8, HLA-DR3, HLA-DQ2, HLA-DRw52