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Understanding Sjögren’s Syndrome: Diagnostic Criteria and Treatment Dosage Guidelines

[Voice of Hope April 2, 2024](Editor: Li Wenhan) Sjögren’s syndrome (SS) is a chronic inflammatory autoimmune disease characterized by lymphocyte proliferation and progressive exocrine gland damage. The main manifestation is that the function of salivary glands and lacrimal glands is damaged, leading to symptoms such as dry mouth and eyes, and may be accompanied by involvement of multiple systems and organs. Here are details about the diagnostic criteria and dosage of treatment medications for Sjögren’s syndrome:

Dry eyes (pixabay)

According to the information in the search results, the diagnostic criteria for Sjögren’s syndrome are mainly based on clinical manifestations, laboratory examinations, and histopathological examinations. The classification standards proposed by the 2023 Chinese version of the Diagnosis and Treatment Standards for Primary Sjögren’s Syndrome and the 2016 ACR/EULAR consensus are as follows:

1. Clinical manifestations:Including symptoms such as dry mouth and dry eyes, which may be accompanied by multi-system involvement, such as skin, joints, lungs, kidneys, nervous system, etc.

2. Laboratory examination:

– The positive rate of antinuclear antibody (ANA) is 50% to 80%, and the positive rate of anti-SSA/Ro and anti-SSB/La antibodies is higher, which are more specific antibodies for diagnosing SS.

– The positive rate of rheumatoid factor is 70%~90%.

3. Histopathological examination:

– Labial gland biopsy shows focal lymphocytic sialadenitis (FLS), that is, a focus of ≥50 lymphocytes in a gland area of ​​4 mm².

– Ophthalmological evaluation of dry eye, including Schirmer test, tear film break-up time (BUT) and corneal conjunctival staining (OSS).

Dry mouth (pixabay)

The dosage of medication to treat Sjögren’s syndrome needs to be determined based on the patient’s specific condition and the severity of symptoms. Here is dosing information for some commonly used medications:

1. Muscarinic agonists:

– Pilocarpine: 5 mg 3 times daily (daily dose 15~20 mg).

– Cevimeline: for patients with moderate to severe glandular impairment but residual salivary gland function.

2. Cyclopentanethione tablets, bromhexine tablets and ambroxol hydrochloride tablets:These drugs can increase the secretory function of exocrine glands, and the specific dosage needs to be adjusted according to the doctor’s prescription and the patient’s response.

3. Immunosuppressants:

-Methotrexate: initial dose is 7.5~15 mg per week, maximum dose is 25 mg/week, can be used in combination with hormones.

– Azathioprine, Mycophenolate mofetil, Tacrolimus, Cyclosporine A: used for the treatment of systemic symptoms, the dosage needs to be adjusted according to the doctor’s guidance and the patient’s condition .

4. Biological agents:

– Rituximab: used alone or in combination with hormones for patients who have failed to respond to treatment.

Renal function changes and related manifestations that may be caused by Sjögren’s syndrome:

1. Renal tubular damage:Sjogren’s syndrome can lead to renal tubular dysfunction, especially in the distal renal tubules. Common manifestations include:

Nephrogenic diabetes insipidus: manifests as polyuria and low specific gravity urine. Patients may have obvious thirst and frequent urination.

Distal renal tubular acidosis (Type 1 RTA): May present with alkaline urine, hypokalemia, and periodic paralysis.

Proximal tubular damage: Less common, but may lead to Fanconi syndrome, which is characterized by increased urinary excretion of various solutes and electrolytes.

2. Glomerular damage:Although less common, Sjögren’s syndrome may also cause glomerulonephritis, which manifests as proteinuria, hematuria, and decreased kidney function.

3. Interstitial nephritis:Sjogren’s syndrome may lead to an inflammatory reaction in the renal interstitium, and renal biopsy may show lymphocyte infiltration and interstitial fibrosis.

Laboratory tests: Monitoring urine routine, urine osmolality, blood electrolytes, blood creatinine, urea nitrogen and other indicators can help evaluate renal tubular and glomerular function.

Kidney biopsy: For patients with obvious clinical manifestations of kidney damage, kidney biopsy is an important means to confirm diagnosis and guide treatment.

Maintain proper hydration (pixabay)

Drug treatment: For patients with renal tubular damage, symptomatic treatments such as electrolyte supplementation, acid-base balance adjustment, and diuretics may be needed. For interstitial nephritis with a significant inflammatory response, steroids and immunosuppressants may be needed.

Supportive care: Maintaining adequate fluid intake, avoiding nephrotoxic drugs, and controlling blood pressure are also important.

The prognosis for kidney damage associated with Sjögren’s syndrome is generally good, and most patients will not progress to end-stage renal disease. However, regular monitoring and appropriate treatment are required to prevent worsening of kidney function.

It should be noted that kidney damage in patients with Sjögren’s syndrome may coexist with other diseases (such as diabetes, hypertension, etc.), so various possible factors need to be comprehensively considered during treatment and management. In addition, since Sjögren’s syndrome is a multi-system disease, treatment also needs to pay attention to the involvement of other systems.

The above drug dosages are for reference only. Actual use must follow the doctor’s prescription and guidance. When treating Sjögren’s syndrome, in addition to drug treatment, attention should also be paid to general treatment and treatment of local symptoms, such as keeping the mouth clean, gargling frequently, and using artificial tears.

Editor in charge: Li Zhi

This article or program was edited and produced by Voice of Hope. When reprinting, please indicate Voice of Hope and include the original title and link.

2024-04-03 06:11:46

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