The relevant expert group of the US Dryness Collaborative Foundation (SSF) has developed guidelines for the treatment of rheumatic disease manifestations of dry syndrome, aiming to improve the quality and consistency of care for patients with dry syndrome by providing management advice, which contains a total of 22 recommendations, mainly including the following: caries prevention; DMARDs in skeletal muscle sarcoidosis in patients with dry syndrome; dry syndrome weakness symptoms The treatment of dry syndrome; the recommendation of biological agents for the treatment of dryness and systemic manifestations of dry syndrome.
I. Caries prevention guidelines
1.For primary dry syndrome, it is recommended to use topical fluoride medication to treat dry mouth syndrome.
2.Although there is no clinical trial to confirm that improving salivary gland function can help prevent dental caries in patients with dry syndrome, it is common knowledge among dentists that increasing salivary volume can reduce the occurrence of dental caries. Based on this theory, we recommend patients to increase salivary gland secretion by taste and chewing stimulation and some medications, such as using sugar-free candy or chewing gum, xylitol, mannitol and prescription medications such as trichothecene and cevimeline.
3.In patients with dry mouth and multiple root caries consider using tooth brightening creams, gels or washes containing chlorhexidine.
4.Remineralization preparations without fluoride can be considered as supplementary treatment for patients with dry mouth and multiple root caries in dry syndrome.
II. Application of DMARDs in skeletal muscle sarcoidosis in patients with dry syndrome
1, Hydroxychloroquine is the first-line drug for the treatment of inflammatory skeletal muscle pain caused by primary dry syndrome.
2.When the treatment of inflammatory musculoskeletal pain in primary dry syndrome with hydroxychloroquine is ineffective, methotrexate alone should be considered.
3.When the treatment of inflammatory musculoskeletal pain in primary dry syndrome with hydroxychloroquine or methotrexate alone is ineffective, the combination of hydroxychloroquine and methotrexate can be considered.
4.When the treatment of inflammatory musculoskeletal pain in primary dry syndrome with hydroxychloroquine combined with methotrexate is not effective, short-term application of glucocorticoid (less than or equal to 1 month), the amount of daily dose should be less than or equal to 15mg. Useful, but hormone replacement drugs should be found as early as possible.
5. If hydroxychloroquine and/or methotrexate or short-term (less than 1 month) glucocorticoids are ineffective in treating inflammatory musculoskeletal pain in primary dry syndrome, consider using leflunomide.
6.If the treatment of inflammatory musculoskeletal pain in primary dry syndrome with hydroxychloroquine and/or methotrexate, glucocorticoids, and leflunomide is ineffective, consider using luzosulfapyridine.
7. If hydroxychloroquine and/or methotrexate, glucocorticoids, leflunomide, or salazosulfapyridine are ineffective in the treatment of inflammatory musculoskeletal pain in primary dry syndrome, azathioprine may be considered. If patients with primary dry syndrome present with major organ involvement, azathioprine may be more appropriate than leflunomide or lorazepam for the treatment of various complications including inflammatory musculoskeletal pain.
8. If treatment of inflammatory musculoskeletal pain in primary dry syndrome with hydroxychloroquine and/or methotrexate, glucocorticoids, leflunomide, azathioprine, or salazosulfapyridine is ineffective, cyclosporine may be considered.
Third, the treatment recommendations for the symptoms of weakness
1.Education on self-care should include recommendations to reduce the manifestation of weakness in dry syndrome through exercise
2. Dehydroepiandrosterone is not recommended to treat the weakness of dry syndrome.
3. Hydroxychloroquine can be used selectively to treat the weakness of dry syndrome
4. Neither etanercept nor liximab therapy is recommended for the treatment of fatigue in dry syndrome.
The following drugs cannot be recommended based on current studies due to insufficient evidence.
Anabolic acid, azathioprine, morte-macrolide, zidovudine, doxycycline, lamivudine, leflunomide, abciximab, belimumab, epalizumab
IV. Suggestions of biological agents for the treatment of dryness and systemic manifestations of dry syndrome
1. Tumor necrosis factor-α (TNF-α) inhibitors should not be used for the treatment of dryness and systemic manifestations in patients with primary dry syndrome.
Note: This recommendation should not be interpreted to discourage the use of TNF-α in the following situations: dry syndrome with concomitant rheumatoid arthritis or if TNF-α is indicated for the treatment of inflammatory arthritis.
2. If anti-TNF-α agents are used to treat dry syndrome overlapping rheumatoid arthritis or other diseases, healthcare workers should consider and monitor the following conditions.
1) Lymphoma and other malignancies (healthcare workers should be aware that patients with primary dry syndrome have an increased risk of developing non-Hodgkin’s lymphoma compared to the general population)
2)Severe infections, including tuberculosis
3)invasive fungal infections
4)Hepatitis B reactivation
5)hepatotoxicity
6)heart failure
7)Hemocytopenia
8)allergy, severe infusion reaction
9)Demyelinating disease
3, Rituximab can be considered for the treatment of dry conjunctivitis in primary dry syndrome and in patients in whom traditional therapies such as topical moisturizers, pro-secretory agents, anti-inflammatory drugs, immunomodulators and teardrop suppositories are not effective.
4, Rituximab can be used to treat patients with primary dry syndrome who have symptoms of dry mouth under the following conditions, including clinicians who determine that there is some evidence of residual salivary secretion in these patients, strong evidence of oral damage, and poor efficacy of traditional therapies including topical moisturizers and pro-secretors.
5. Rituximab may be used to treat primary dry syndrome and any or all of the following systemic disease manifestations.
1) Cryoglobulinemic vasculitis
2)Vasculitis
3) severe parotid swelling
4)inflammatory arthritis
5)Pulmonary related disease
6) Peripheral neuropathy – especially mononeuritis
6. Patients and health care workers should be aware that although serious adverse reactions to rituximab are uncommon, caution and observation should be exercised for the following conditions that occur with rituximab.
1) Infusion reactions
①Non-Hodgkin’s lymphoma (NHL) with tumor lysis syndrome
②Progressive multifocal leukoencephalopathy (PML)
③Hepatitis B reactivation with possible fulminant hepatitis
④Severe skin mucosal reactions
2) Infection
①Intestinal obstruction and perforation
②Cardiac arrhythmia and angina pectoris
3) Hematocrit
①Severe bacterial, viral or fungal infections.
②Risks and benefits must be carefully considered during pregnancy and lactation.
③When patients are on rituximab, vaccination should be avoided.