How is Sjogren's nerves treated symptomatically? An article to understand the symptoms, diagnosis, treatment of the latest review

Edited and sorted out by Yimaitong, please do not reprint
without authorization.

Sjogren syndrome (SS) is a complex autoimmune rheumatism with primary SS (pSS) referring to the diagnosis of SS in the absence of other autoimmune diseases, with a population prevalence of 0.
1%-3%.

pSS can affect the perinervous, central, and autonomic nervous systems, with the peripheral nervous system being affected much more frequently than the central nervous system
.
There are no valid criteria for diagnosing or classifying neuropsychiatric manifestations
of pSS.
This article reviews the clinical manifestations, evaluation, and treatment
of the pSS nervous system.

Clinical manifestations

There are wide differences
in neurological performance of pSS.
There are significant heterogeneity in the peripheral nervous system, manifested as painful sensory peripheral neuropathy, multiple mononeuropathy, etc.
, of which autonomic neuropathy and radiculopathy are rare; Central nervous system manifestations include aseptic meningitis, headache, cognitive impairment, etc
.
Specifically, please refer to Table 1
.

Table 1 Clinical manifestations of the pSS nervous system

Evaluation and diagnosis

Diagnosis of pss-related peripheral nervous system manifestations should be combined with clinical presentation and laboratory tests, and patients should be evaluated for sensory, motor, and autonomic symptoms
.
Laboratory tests should begin with nerve conduction studies and electromyography (EMG) and assess whether nerve conduction abnormalities are limited to sensory fibers and are symmetrical or asymmetrical
.
Neuroimaging can also assist in diagnosis, for example, spinal cord MRI can identify T2 high-intensity lesions and thickened nerve roots in patients with sensory neuropathy and radiculopathy
, respectively.
Quantitative sensory testing or intraepidermal nerve fiber densitometry is an ideal method
for diagnosing small fibrous neuropathy.

Patients with pSS with central nervous system presentation should be evaluated
with cerebrospinal fluid and brain imaging.
When central nervous system lesions are active, CSF may present with elevated lymphocyte and protein levels, elevated IgG index, and oligoclonal bands
in electrophoresis.
In 80% of patients with neuropsychiatric symptoms with pSS, MRI results are normal and subcortical nonspecific white matter lesions may also be present, manifested by microvascular disease
.

Single-photon emission computed tomography (SPECT) shows hypoperfusion in patients with pSS with neuropsychiatric symptoms and normal MRI, and although a nonspecific result, cortical hypoperfusion is associated
with neuropsychological evaluation in patients with pSS and cognitive dysfunction.

treat

Peripheral nervous system lesions

Treatment in patients with small fibrous neuropathy is to relieve symptoms and is usually treated
with gabapentin (gabapentin or pregabalin) or with serotonin-norepinephrine reuptake inhibitors (duloxetine or venlafaxine).
Tricyclic antidepressants are also effective but may worsen dry
mouth.
Treatment of pSS-associated sensory neuropathy is aimed at preventing long-term disability, with immunomodulators commonly used, most commonly in combination with glucocorticoids (intravenous shock) and intravenous immunoglobulin (IVIG
).
Refractory patients may choose intravenous cyclophosphamide (IV) in combination with glucocorticoids
.
Oral immunosuppressants are commonly used to induce hormone replacement therapy
after remission.
A key issue in the treatment of pSS-related sensory neuropathy is the timing of medication, and some scholars believe that the optimal treatment window is within
8 months after the onset of the disease.
Treatment of multiple mononeuropathy is similar to vascular neuropathy, using intravenous glucocorticoids and / or cyclophosphamide
.
IVIG has been recommended as a first-line treatment
for radiculopathy.

Lesions of the central nervous system

First-line treatment of acute or rapidly progressive central nervous system disease is high-dose glucocorticoids (methylprednisolone 1 g/d IV for 3 to 5 days, followed by prednisone 1 mg/kg/d IV or orally).

The second-line treatment in the presence of inflammation of the lesion is cyclophosphamide IV (700 mg/^m2) for 6 months
.
Azoprine and mycophenolate mofetil have been reported to treat central nervous system lesions, and cases have been reported to reduce the frequency and severity
of migraine attacks in patients with pSS treated with intrathecal rituximab for refractory headaches.

In addition, the cornerstone of the treatment of autonomic neuropathy is symptomatic treatment (especially for orthostatic hypotension
).

conclusion

The neuropsychiatric symptoms of pSS are associated with severe disability and low quality of life and should be diagnosed and treated early, while identifying the patient's immunological and imaging features will help explore its pathogenesis
.
Existing cohort studies have highlighted the importance of neuropsychiatric symptoms of pSS in the development and increase of disability, and further clinical trials and studies
are needed to identify the incidence and prevalence of neuropsychiatric manifestations of pSS, the risk factors for their occurrence and recurrence, and to standardize their diagnostic and/or classification criteria.

Reference: Appenzeller S, Andrade de Oliveira S, Bombini MF, Sepresse SR, Reis F, Cavalcante França Junior M.
Neuropsychiatric manifestations in primary Sjogren syndrome[J].
Expert Rev Clin Immunol.
2022 Aug 24.
doi: 10.
1080/1744666X.
2022.
2117159.
Epub ahead of print.
PMID: 36001085.