ORADE Grant Recipient – Dr. Mary Bell

Dr. Mary Bell was the recipient of a 2017 ORADE grant. Below is a summary of her learnings.

ORADE supported my attendance at the 2017 ACR Sate-of-the-Art (SOTA) Clinical Symposium in Chicago, Illinois April 29-30. This accredited symposium is well organized and addresses a wide range of topics.  The speakers are a mix of local and world experts in the field of Rheumatology. The syllabus is electronic and easy to navigate. The pre-test and post-test allow one to identify personal learning gaps for self-directed CME.

New insights in Sjogren`s Syndrome (SS) include: there is an X-chromosome dose effect, autoantibodies may be present up to 18 years before diagnosis, polyautoimmunity is common and similar in primary and secondary Sjogren`s, the lymphoma risk has been quantitated at 6-9X the general population, and mortality is not increased over the general population (with the exception of those with low C4, cryoglobulinemia, and lymphoma). New ACR-EULAR criteria have been published and a score of 4 or greater required for classification (+labial biopsy = 3, + Anti-SSA = 3, ocular surface staining = 1, Shirmer 5 or less = 1, and salivary flow 0.1 ml per min or less = 1).  Salivary gland ultrasound (US) can detect SS with sensitivity 52-95% and specificity 84-99% and may become a diagnostic tool in the future. Two kinds of lymphoma are seen in patients with SS. MALT (younger patients, disease of shorter duration, and localized) and diffuse large B-cell lymphoma (older). Five year survival is better in MALT (90%) than DLBL (58%).  Clinical trials are ongoing with anti-BAFF, anti-CD40, P12K delta inhibitor, cathepsin S inhibitor, LD IL-2, anti-IL-6, and anti CD28.  Rituximab is looking promising, especially in those with high parotid gland CD20 cell numbers. Disease activity and disease burden can be quantified using 2 new indices, ESSDAI and ESSPRI.

Two new antibodies can detect necrotizing myositis (NM)– anti-HMGCR and anti SRP. IVIG is effective as first line treatment of statin-associated autoimmune NM. Amyopathic dermatomyositis can be detected by anti-SAE and anti-MDA-5  testing. Autoantibodies associated with dermatomyositis include: anti-NXP-2, anti-TIF-1g, and anti-Mi-2.

In the Hopkins Cohort, headache is not increased in SLE over the general population. Psychosis is less common than in the past but cognitive impairment is frequent (80%) at baseline and improves over time. Depression is common (20%) and correlates with cognitive impairment and prednisone use. Risk for stroke in SLE include: APS and high cholesterol, recent herpes zoster infection, and vasculitis.

Vascular EDS patients are at high risk of arterial aneurysm and rupture occurring 66% in the thorax and abdomen, 17% head and neck, and 17% in the extremities. Advise patients to wear a MedicAlert bracelet indicating vascular EDS on it. Twenty-five percent have a major complication by age 20, 80% with a major complication by age 40, and median age of death age 50.  Pregnancy complications (prematurity, vaginal and perineal tears, uterine prolapse and rupture, and arterial dissection) in this population are common and there is a 5-15% risk of maternal death with each pregnancy. Avoid muscle relaxants and opioids; collision sports, heavy lifting and weight training; arteriography due to risk of vascular injury; and routine colonoscopy.

In summary:

  • New classification criteria in Sjogren`s Syndrome
  • Salivary gland US is now being used
  • Lymphoma in SS – MALT and diffuse large B cell
  • Test for statin-induced necrotizing myopathy with anti-HMGCR and treat with IVIG
  • Vascular EDS have major pregnancy complications and high risk of death