Maternal Sjögren syndrome

An autoimmune inflammatory disease affecting the salivary glands, lacrimal glands and other moisture producers that causes irritation and dry mouth and eyes. It can also affect joints, skin, vagina, lung, muscles, liver or kidney (MedlinePlus 2017).
It can be primary or secondary to other inflammatory autoimmune diseases such as rheumatoid arthritis, lupus or scleroderma.

It is much more common (90%) in women. It may worsen (10% of cases) during pregnancy and 5% of newborns may have heart block (Priori 2013).

Like other inflammatory autoimmune diseases, it may be accompanied by Raynaud's Phenomenon in hands (Isenberg 1995), but not usually in nipples.

There may be mild hyperprolactinemia (Jara 2011, Gutiérrez 1994) which has not been found in other observations (Karakus 2017).

The mammary gland is not affected. Normal breastfeeding has been observed in women affected by this disease, with 32% of women still breastfeeding at 6 months and 8% at one year (Priori 2013).

The required treatment is usually topical (artificial tears, ocular and vaginal lubricants, oral salivation stimulants) and are compatible with breastfeeding. If other inflammatory diseases coexist, refer to the required medication.

Expert authors do not contraindicate breastfeeding (Lawrence 2017 p.595).

• Azathioprine (Very Low Risk)
• Cyclophosphamide (Very High Risk)
• Hydroxychloroquine Sulfate (Very Low Risk)
• Hypromellose (Very Low Risk)
• Ibuprofen (Very Low Risk)
• Maternal Connective Tissue Disease: Rd; Arthritis; Lupus (Low Risk probable)
• Maternal Rheumatoid Arthritis (Low Risk probable)
• Methotrexate (Low Risk probable)
• Mycophenolate Mofetil (High Risk probable)
• Non-Hormonal Vaginal Lubricant (Very Low Risk)

Synonyms

• Keratoconjunctivitis sicca
• Sicca syndrome
• Xerostomia

Writings

• Σύνδρομο Sjögren μητέρας (Greek)