Progress in the treatment of dry syndrome

  Dry syndrome is a chronic inflammatory autoimmune disease that mainly invades salivary and lacrimal glands. The prevalence of this disease in China is 0.29%~0.77%. 2012 American College of Rheumatology new consensus no longer distinguishes dry syndrome as “primary” and “secondary”, because both have the involvement of autoimmune mechanism, the distinction is easy to cause confusion of disease diagnosis. There are many criteria for classifying dry syndrome.  The classification criteria of dry syndrome are many, from the San Diego criteria and FOX criteria in 1986, to the European criteria and American College of Rheumatology diagnostic criteria in 1993, to the international standard in 2002 and the new classification criteria of dry syndrome promulgated by ACR in 2012, each of these criteria has its own characteristics. 2012 ACR criteria are: for people with suspected symptoms, if two of the following three items are met Positive anti-SSA and/or anti-SSB, or (RF positive and ANA ≥ 1:320); lower lip biopsy: focal lymphocytic salpingitis with ≥ 1 lymphocytic foci in 4 mm interstitial tissue; dry keratoconjunctivitis with a corneal staining score ≥ 3 (no current daily glaucoma eye solution, no keratoplasty or cosmetic eyelid surgery in the past 5 years), and excluding amyloidosis, IgG4-related disease, history of cervical head and facial radiation therapy, hepatitis C, AIDS, lymphoma, nodal disease, and transplantation-resistant host disease. The sensitivity of the diagnosis is 93% and the specificity is 95%.  The general treatment of dry syndrome includes rest, adequate sleep, avoiding overexertion and smoking and alcohol cessation, maintaining a certain level of humidity in the room, preventing upper sensation, strengthening symptomatic management such as the use of bromhexine tablets, ambroxol and artificial eye drops, using non-steroidal anti-inflammatory drugs if there is arthralgia, intravenous potassium supplementation if there is hypokalemic paralysis, oral potassium supplementation after remission, hormones and modification of the condition if there is severe organ involvement and high risk of lymphoma Anti-rheumatic drugs such as hydroxychloroquine  It has been found that the use of M3 agonist pilocarpine (furfurylated brassin) 5mg 4 times a day can increase the rate of saliva secretion by 20-40%, but there are often sweating and gastrointestinal intolerance side effects, while the new drug Evoxac (cevimeline) is more effective for dry mouth and eyes, especially dry eyes, the effect lasts longer and has fewer side effects. IFN-α is effective in those with less severe disease (especially those with incomplete loss of lacrimal or salivary gland function), while immunosuppressive agents (azathioprine and cyclosporine) have limited effect on dry mouth and eyes.
α administered via the oral mucosa for dry mouth may be effective.  Oral hormones may be used in cases with systemic damage such as interstitial lung fibrosis, glomerulonephritis, neurological damage, hepatic damage, hematocrit and myositis; hyperglobulinemic purpura; necrotizing vasculitis, extensive lymphoproliferative and persistent, recurrent enlargement of the parotid glands. Plasma exchange is feasible for hyperglobulinemia and renal tubular acidosis. TNF-α antagonists have been shown to be ineffective. B-cell targeting holds some promise, with 2 small clinical trials finding improvements in certain secondary endpoints (social function SF-36, ESR and RF titers) with Rituxan compared to baseline. Whether anti-BAFF belimumab for lupus is effective in dry syndrome is unclear.  In conclusion, the prognosis of dry syndrome is better for those with slow development of the disease course and only salivary and lacrimal gland lesions, and worse for those with severe organ damage or complications of malignancy, which should be diagnosed early, treated actively, and followed up regularly.