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Yimaitong edited and sorted, please do not reprint
without authorization.
Systemic sclerosis (SSc) is an autoimmune disease characterized by manifestations such as pulmonary
It has recently been found that fibrosis of the small and large salivary glands, which are directly related to the pathogenesis of SSc, may be the main cause
of xerostomia in patients with SSc.
The literature on Sjogren syndrome (SS) is mainly based on primary SS (pSS), and there are few studies on SSc-related SS, but oral and facial symptoms may directly affect the quality of life of patients, and it is important
to pay attention to such neglected issues in the overall management of patients with SSc.
This article will review the clinical manifestations, diagnosis, treatment and future prospects
of SSc-related SS based on the current status of diagnosis and treatment of pSS.
Diagnostic methods
1.
Screening and clinical assessment
Clinical screening is an important step
in patients with xerostomia.
The Systemic Sclerosis Oral Disorder (MHISS) scale can be used to screen
for xerostomia.
Saliva determination is one of the criteria for judging dry mouth, and saliva flow above 0.
1ml/min is normal
.
Dry eye is another characteristic clinical manifestation of SS, and patients may present with burning or painful eyes and increased
sensitivity to light.
The Schirmer test is a method to test the function of tear secretion (standard Schirmer filter paper is left on the edge of the patient's lower eyelid for 5 minutes and removed, the filter paper is soaked < 5 mm positive), the application of corneal fluorescein, tiger red or Lisimine green staining, and the assessment of tear
of dry eye.
2.
Histological evaluation of small salivary
Sialadenitis based on histologic diagnosis of salivary glands is a typical pathological manifestation
of SS.
The revised 2016 American College of Rheumatology (ACR)/European Union Against Rheumatism (EULAR) criteria proposes that small salivary gland biopsy (mSGB) is one of the key criteria for the diagnosis of
pSS.
In addition, excessive deposition of extracellular matrix by fibroblasts can cause tissue fibrosis, which is involved in the pathogenesis of SSc, so small salivary gland fibrosis can distinguish SSc-associated SS from pSS.
However, mSGB is an invasive test with a risk
of adverse events.
3.
Imaging evaluation of major salivary gland involvement
As one of the auxiliary examinations for the diagnosis of pSS, ultrasound has the advantages
of low cost, non-invasive, and no radiation.
Ultrasound is more sensitive to the diagnosis of pSS than salivary gland contrast and salivary salivary scopy, but the role of ultrasound in assessing primary salivary gland involvement in SSc remains unclear
.
Specific MRI sequences to identify parotid fatty deposits (MR salivary gland angiography, STIR, T2–3D-DRIVE) and peripheral ductal dilation have shown good diagnostic performance in pSS, but their role in SSc remains to be determined
.
In addition, a chest HRCT scan is recommended for initial screening for SSc-associated
with SSc-ILD.
Treatment
Non-pharmacological treatments
Nonpharmacologic treatments are the basis for
managing SS.
Patients should be instructed to reduce alcohol or spice intake, as they can lead to dry
mouth.
In addition, clinicians should consider discontinuing drugs with anticholinergic properties (antidepressants or antihistamines), which may induce or worsen
SS.
Taste stimulants (sugar-free acid candies, lozenges, and
.
drug therapy
There are currently no pharmacological studies
for SSc-related SS.
Existing treatments can be divided into topical replacement therapy (artificial tears, artificial saliva) and drugs
that increase aqueous secretions after stimulation of muscarinic receptors (
The 2019 European Alliance Against Rheumatism (EULAR) recommendations for the management of pSS emphasize that topical symptomatic treatment should be preferred because of the minimal adverse effects; Saliva therapy is also recommended for patients who do not respond to non-pharmacological therapy
.
and oral care for patients with SSc with saliva removal therapy (pilocarpine tincture 40 drops/day; pilocarpine 5 mg, 3 tablets / day;
.
B cells are involved in the pathogenesis
of SSc and pSS.
.
Other biologics targeting B cells, such as
in patients with SSc.
A study evaluating the effect of autologous adipose tissue injection on SS symptoms and MHISS parameters in patients with SSc showed significant improvements
in SS symptoms (p<0.
001), MHISS score (p=0.
003), and self-completed dry mouth scale score questionnaire at 6 months postoperative compared with baseline.
conclusion
While pSS is reported by less than 20% of patients with SSc, up to 80% may have subjective SS symptoms
.
The incidence of SSc-associated SS is high, but there are still few studies on it, and the inclusion of SS symptoms in the outcome assessment of SSc randomized controlled trials may help highlight this neglected problem
.
Although patients with SSc and pSS tend to have a milder phenotype in visceral manifestations such as ILD or PAH, they are at higher risk of
to identify pSS in SSc.
In addition to the association with pSS, histological findings suggest that microsalivary gland fibrosis in SSc patients may contribute to a high incidence
of SSc-associated SS.
In addition, the role of non-invasive diagnostic instruments, such as ultrasound evaluation of the main salivary glands, in SSc remains to be determined
.
In terms of treatment, non-pharmacological treatment is the basis for the management of SS, and further drug research
for SSc-related SS is expected.
References: Zimmermann F, Robin F, Caillault L, et al.
Sicca syndrome in systemic sclerosis: a narrative review on a neglected issue[J].
Rheumatology (Oxford).
2022 Jul 22:keac412.
doi: 10.
1093/rheumatology/keac412.
Epub ahead of print.
PMID: 35866609.
