Sjögrens Forum

Q: I am a 34 year old 15 week pregnant female with a 2 year history of UCTD diagbosis by my rheumatologist due to severe morning stiffness, photosensitivity, Raynaud's Phenomenon, chronic mouth ulcers, joint pain, and fatigue. Gastroparesis diagnosis May 2009 (abnormal gastric emptying scan) with severe bouts of nausea and vomiting, plus chronic sinus issues with severe post nasal drip and use saline, netty pot, humidifier, meds to try and control symptoms.

Mutiple positive ANA 1:640 speckled/homogenous pattern

High C4

High ESR

High CRP 10.5

Positive SSB antibody 1.6 (range 0-9)-recent finding

Low Vitamin B12 and low iron.

All other laboratory tests are normal.

Would this constitute primary SJS or secondary with Lupus?  I have dry eyes (not severe) diagnosis by an eye doctor and have a growth on my right eye called a pytergium and was given Sooth XP eye drops.  When not pregnant I take Plaquenil for my

UCTD (Undifferentiated Connective Tissue Disease).  I read that the criteria for UCTD would no longer be considered undifferentiated if SSB antibody is positive, so I am wondering what this means (SJS or Lupus) and my risk for neonatal lupus/congenital heart block?  I am being monitored closely by my rheumatologist and high risk obstetrician and have one healthy baby born in August of 2008.  Thank you for any advice, I truly appreciate it.

A: Unless you have had high RNP Abs (Ribo  nuclear protein antibodies), your history/symptoms fit more with mild primary SLE with probable secondary Sjs. La(SSB) Ab without Ro(SSA) would fit more with SLE than Sjs. Your age fits more with SLE than primary Sjs but there is a blurred line between these disorders. After your delivery, you may consider participating in one of the SICCA Registries where they would do a complete Sjs evaluation. If the salivary gland biopsy showed lymphocytic infiltration, that would add to your understanding of your symptom complex-but not likely change your treatment or prevention options. SICCA Registry in US (UCSF (San Francisco, CA), Johns Hopkins (Baltimore, MD), Penn (Philadelphia, PA).

Being followed closely by a rheumatologist and high-risk obstetrician and with a normal prior pregnancy-you should be fine.

Elevated C4 and ESR can occur during pregnancy and is seen Not as hallmark for clinically significant inflammation.

With gastrointestinal motility problems and decreased B12 and iron without source of blood loss, I would suggest malabsorption or a gastrointestinal immune process. Absorption of antibodies to gastric/parietal cells (cells in the stomach) can occur. You could consider taking sublingual B12 (over the counter) and/or liquid iron (better tolerated)

Being followed closely by a rheumatologist and high-risk obstetrician and with a normal prior pregnancy-you should be fine.

Assume your Vitamin D was tested prior to pregnancy. Low Vitamin D is common in autoimmune disorders, and replacing it to help decrease inflammation is currently recommended.

For more information on congenital heart block, you may find Jill Buyon's work of interest. www.med.nyu.edu/medicine/labs/buyonlab/.../heart.html

Also- google books - Lupus:molecular and cellular pathogenesis (Kammer/Tsokos ed) Autoimmune-Associated Congenital Heart Block by Jill Buyon Capter 30, p 492

Dr.c