Clinical manifestations of Sjogren's syndrome

Sjogren's syndrome (Sjogren syndrome) is a chronic inflammatory autoimmune disease
characterized by lymphocyte proliferation and progressive exocrine gland damage.
In addition to impaired salivary gland and lacrimal gland function, multi-system multi-organ involvement may also occur, and autoantibodies and hyperimmunoglobulinemia
may exist in serum.
SS is divided into secondary S S and primary SS (primarySS, pS S) according to whether it is accompanied by other connective tissue diseases, the former often secondary to hand systemic lupus erythematosus, rheumatoid arthritis, etc
.
pSS is a global disease, the prevalence in the Chinese population is 0.
3%~0.
7%, more common in women, the male to female ratio is 1:(9~20), the age of onset is mostly 40~50 years old, and it can also be seen in children
.
Theexact etiology and pathogenesis of p S are unknown, and it is currently believed to be immune dysfunction caused by genetics, viral infections, sex hormone abnormalities and other factors
.
 

The disease has a hidden onset, and the clinical manifestations are not
severe.
Some patients only have local symptoms of dry mouth and eyes, and they present to stomatology and ophthalmology, while some patients are characterized by systemic damage
.

Local manifestations

Dry mouth

Caused
by decreased saliva secretion and deficiency of salivary mucin.
Patients often drink water frequently, often need water when eating dry food, and severe cases may have difficulty eating, flaky tooth loss and multiple rampant dental
caries.
40%~50% of patients can have salivary gland enlargement, recurrent attacks, usually without fever, which can be distinguished from mumps
.
If the gland continues to enlarge and appears nodular, it is necessary to be alert to malignant lesions
.

Dry eyes

Caused
by hyposecretion of lacrimal glands.
Patients have dry eyes, frosty feeling, eye congestion, severe cases can have complications such as keratoconjunctivitis dry, corneal epithelial erosion, corneal neovascularization and ulcer formation, and even corneal perforation and blindness
.

System performance

Systemic impairment occurs in about two-thirds of patients, and some patients have systemic symptoms
of fatigue and fever.

skin

Skin lesions in pSS include dry skin, Raynaud's phenomenon, and vasculitis
.
About 10% of pSS have cutaneous vasculitis, purpura is the most common, and skin ulcers, gangrene, pitting scars, microinfarcts, urticarial lesions, periungual infarction, and nontender erythema can also occur
.

Muscles and joints

Joint symptoms can occur in about 50% of patients with p SS, usually presenting as chronic, recurrent, symmetric arthralgia, and arthritis occurs in only about 10% of patients, and erosive arthritis is rare
.
 

PSS may present with muscle involvement, but symptoms of muscle weakness require differentiation for fibromyalgia syndrome, hormone-related, and other concurrent conditions
.
Serum creatase, electromyography, and muscle MR! contribute to the diagnosis
of PSS-associated myositis.

respiratory system 

Respiratory involvement of pSS is characterized by dry airways, interstitial lung lesions, but also hairy bronchitis and bronchiectasis, and rare manifestations include amyloidosis, granulomatous lung disease, pseudolymphoma, pulmonary hypertension, and pleural lesions
.
10%~20% of the elderly can have pSS-related pulmonary interstitial lesions, and the pathological type is often manifested as non-specific interstitial pneumonia (NSIP), common interstitial pneumonia (UP) and lymphocytic interstitial pneumonia (LIP).

 

digestive system 

Patients with pSS often have symptoms of gastroesophageal reflux disease (GERD), which is due to reduced saliva flow that does not naturally buffer the acidic contents of the reflux
.
In addition, the use of NSAIDs and glucocorticoids predisposes patients to gastritis and peptic ulcers
.
Some patients with GERD present with laryngotracheal irritation
.
25% of patients have liver function damage, elevated aminotransferases, and even jaundice, and occasionally hepatosplenomegaly
.
Patients with pSS should be alert to primary cholangitis (pBC) when serum alkaline phosphatase levels are elevated
, often with both.
pSS can cause exocrine pancreatic dysfunction, and its pathological mechanism is similar to salivary gland involvement, mainly due to lymphocyte infiltration leading to pancreatic alveolar atrophy, pancreatic duct stenosis and other chronic pancreatitis changes
.

kidney

4%~30% of pSS fools may have renal damage, the most common is tubulointerstitial nephritis, and glomerulonephritis and interstitial cystitis
may also occur.
Patients with renal interstitial lesions may be clinically manifested as renal tubular acidosis, nephrogenic diabetes insipidus, Fanconi syndrome, renal calcification/stones, etc
.
Renal puncture
is recommended if available.
 

nervous system 

The neurologic manifestations of pSS are diverse, and peripheral, autonomic, and central nervous system can be affected
.
Among them, peripheral neuropathy is the most common, 10%~20% of patients can have peripheral neuropathy, mostly manifested as symmetric peripheral sensory nerve involvement, often occur in patients with hyperglobulinemia purpura, motor nerve involvement can also occur
together.
Autonomic syndrome is a more serious small-fiber neuropathy manifested by orthostatic hypotension, Adie pupil, anhidrosis, tachycardia, and bowel dysfunction
.
Central nervous system lesions of pSS can present with asymptomatic and furuncle encephalopathy, as well as neuromyelitis optica spectrum disease or myelopathy in progressive transverse myelitis
.

Blood system

PSS can cause autoimmune cytopenias, of which leukopenia is the most common, followed by immune thrombocytopenia
.
The risk of developing lymphoma is 18.
9 times that of the normal population, most commonly B-cell lymphoma
in the extranodal marginal area of mucosa-associated lymphoid tissue (MAIT).
 

Cryoglobulinemia

pSS-associated cryoglobulinemia is associated with long-term activation of B cells, and the risk of lymphoma is increased, and cryoglobulin-associated vasculitis and membranous glomerulonephritis may occur, with a poor prognosis
.
The type is usually a mixture of type II and III cryoglobulinemia
in the later tense.

In short, because Sjogren's syndrome has a more insidious onset, the clinical manifestations vary
in severity.
Familiarity with the clinical manifestations of Sjogren syndrome is important for identifying patients, evaluating their condition, and effectively treating them
.