Summary of clinical manifestations of Sjogren's syndrome

Sjogren's syndrome (pSS) is a chronic inflammatory autoimmune disorder characterized by lymphocyte proliferation and progressive exocrine gland damage, in which a variety of autoantibodies
are present in the blood serum.
In addition to impaired salivary glands and lacrimal glands, multi-organ and multi-system involvement
may occur.

pSS mostly has an insidious onset and clinical manifestations vary
.
Some patients only have local symptoms of dry mouth and eyes, and seek treatment in stomatology and ophthalmology, while some patients have damage to important organs as the first symptom
.
More than 80% of patients experience dryness, fatigue, and pain
.

Local manifestations

Dry mouth

Caused
by decreased saliva secretion and lack of salivary mucin.
Patients drink water frequently, often need water to take when eating dry, severe cases may have difficulty eating, tooth chip loss and multiple caries
.
Patients may have salivary gland enlargement with recurrent episodes without fever
.
If the gland is persistently enlarged and nodular, it is necessary to be vigilant for malignant lesions
.

Dry eyes

Caused
by low secretory function of the lacrimal glands.
Patients with dry eyes, scrubbing and congestion, severe cases may appear dry keratoconjunctivitis, corneal epithelial erosion, corneal neovascularization and ulcer formation, and even corneal perforation, blindness
.

System performance 

About one-third of patients may have systemic damage, and a small number of patients have systemic symptoms
such as fever and lymphadenopathy.

skin

pSS has dry skin, Raynaud's phenomenon, and cutaneous vasculitis
.
The latter is most commonly
seen with double lower extremity purpura.
Others have urticaria-like skin lesions, erythematous nodules, etc

Joint muscles

About 50% of pSS can have arthralgias, which is chronic, recurrent, and involves the hand joints, and only 10% of patients develop arthritis, while erosive arthritis is rare
.
Myalgia and myasthenia should be differentiated for fibromyalgia, hormone-related myopathy, hypokalemia due to secondary renal tubular acidosis, or other intercurrent conditions
.

Serum creatine kinase, serum potassium and electromyography, and muscle magnetic resonance imaging (MRI) help confirm and differentiate
pSS-related myopathy.

respiratory system

Respiratory involvement is mainly due to dry airways, interstitial lung lesions, bronchiolitis, bullae, and bronchiectasis
.
Rare manifestations are amyloidosis, pseudolymphoma, pulmonary hypertension, and pleural lesions
.
Pulmonary interstitial lesions are the most common, and the pathological types are different, including non-specific interstitial pneumonia (NSIP), lymphocytic interstitial pneumonia (LIP), interstitial pneumonia vulgaris (UIP) and organic pneumonia (OP), These types present different features
on high-resolution chest CT.
Interstitial lung disease is one of the leading causes of
death from pSS.

digestive system

Patients with pSS often have symptoms of gastroesophageal reflux disease, some of which present with laryngotracheal irritation, which is related to
reduced saliva flow and acidic gastric contents that cannot naturally buffer reflux.
In addition, the use of NSAIDs and glucocorticosteroids can lead to the development of gastritis and peptic ulcer
in patients.
Twenty-five percent of patients had liver damage, elevated aminotransferases, and even jaundice, with some primary biliary cholangitis (PBC).

pSS can cause exocrine pancreatic dysfunction, and its pathological mechanism is similar to salivary gland involvement, mainly due to chronic pancreatitis changes
such as pancreatic acinar atrophy and pancreatic duct stenosis caused by lymphocyte infiltration.

kidney 

The most common renal damage in patients with pSS is tubulointerstitial lesions, and patients with renal interstitial lesions can be clinically manifested as renal tubular acidosis, nephrogenic diabetes insipidus, Fanconi syndrome, renal calcifications/stones, etc.
, and some patients have periodic paralysis due to hypokalemia
.
A small number of patients develop glomerulonephritis and interstitial cystitis
.
Renal puncture is recommended to determine the nature of the lesion and the degree of
activity.

nervous system 

Neurological involvement in pSS is varied, including peripheral, autonomic, and central nervous system involvement
.
Peripheral neuropathy is the most common (10%~20%), mostly symmetric peripheral sensory neuropathy, common in patients with hyperglobulinaemic purpura, motor nerve involvement can also co-occur
.
Autonomic syndrome is manifested by orthostatic hypotension, Adie pupil, anhidrosis, tachycardia, gastrointestinal dysfunction, etc
.
Small-fiber neuropathy often causes paresthesias such as a burning sensation
.
Central nervous system lesions are uncommon and often manifest as leukoencephalopathy, neuromyelitis optica spectrum disease, or transverse myelitis
.

Blood system

Cytopenias may occur, with mild leukopenia being the most common
.
Thrombocytopenia is often a difficult treatment for rheumatologists, and some patients are stubborn, prone to recurrence, and difficult to control
.
The risk of lymphoma is several times higher than in healthy people, most commonly mucosa-associated marginal zone B-cell lymphoma (MALT).

Cryoglobulinemia

Manifests as cryoglobulin-associated vasculitis and membranoproliferative glomerulonephritis
.
Associated with long-term activation of B cells, the risk of lymphoma is increased and the prognosis is poor
.
It is usually characterized by mixed cryoglobulinemia
in which both type II and type III cryoglobulins are present.
 

Autoimmune thyroid disease 

Often accompanied by pSS, including Graves disease and Hashimoto thyroiditis, some patients may have hyperthyroidism or hypothyroidism, and autoantibodies against thyroid antigens can be detected in the blood
, including thyroid globulin antibodies and thyroid microsomal antibodies or thyroid-stimulating receptor antibodies.

In short, pSS as a systemic disease, in addition to glandular involvement, there will be many extraglandular manifestations, such as systemic lymphadenopathy, kidney involvement, central nervous system, lung, joints, skin, muscle and peripheral neuropathy
.

References:

[1] Zhang Wen, Li Xiaomei, Xu Dong, etc Diagnosis and treatment of primary Sjogren's syndrome[J].
Chinese Journal of Internal Medicine, 2020, 59(4): 269-276 pages