Sjögren's Syndrome Nervous System Damage

Introduction to nervous system damage in Sjogren's syndrome Sjogren's syndrome (Sjögren's syndrome, SS) is a chronic inflammatory autoimmune disease mainly caused by exocrine gland damage. In 1892, Micklicz first reported the disease. In 1933, Sjögren carried out more detailed The study, also known as Micklicz-Sjögren syndrome. The disease is caused by lacrimal gland and parotid gland damage and inflammatory cell infiltration, mainly manifested as dry mouth, dry eyes, and can show multiple systemic and multi-organ damage, and can be associated with other connective tissue diseases such as rheumatoid arthritis, systemic Lupus erythematosus, dermatomyositis, scleroderma, etc. overlap, SS often combined with various damages of the nervous system, and even the main clinical manifestations of neurological damage, such as peripheral neuropathy, myopathy. basic knowledge The proportion of illness: 0.005% Susceptible people: no specific population Mode of infection: non-infectious Complications: systemic lupus erythematosus dermatomyositis scleroderma

Sjogren's syndrome nervous system damage etiology

(1) Causes of the disease

The etiology of SS has not yet been clarified, and it has been recognized that SS is an autoimmune disease in which an exocrine gland is the main target organ.

(two) pathogenesis

There are three possibilities for the pathogenesis of Sjogren's syndrome:

1. Genetic or congenital immune system abnormalities, manifested as excessive spontaneous activity of B cells, may be related to excessive TH cell function or TS cell function.

2. Acquired antigens (such as viral infections) alter autoantigens and cause autoantibody production.

3. Exogenous antigens interact with specific genetic susceptibility, resulting in excessive B cell reactivity and lymphocyte infiltration of target organs, and immune complexes may also be involved in the pathogenesis.

According to reports in the literature, SS with neurological damage may be related to vasculitis. It is believed that ischemia caused by vasculitis is the main cause of nerve damage. In SS with peripheral nerve damage, peripheral nerve biopsy or autopsy has been confirmed. Acute or chronic vasculitis and/or perivascular inflammation, the disappearance of myelinated fibers, vascular inflammatory changes in the adventitial arterioles, destruction of the vessel wall, and significant narrowing or occlusion of the lumen.

Patients with SS and central nervous system damage, combined with peripheral vasculitis, have also confirmed extensive vasculitis in their brain autopsy. In addition, some scholars believe that SS with neurological damage may be related to inflammatory cell infiltration, brain parenchyma Or chronic inflammatory cell infiltration in the peripheral nerve sheath may be the cause of neurological damage. Others believe that there may be anti-neuronal autoantibodies, which are related to the central nervous system damage of SS, and are thought to be stimulated by immune abnormalities. Vasospasm may also be the cause of transient single or multifocal symptoms that cause damage to the central nervous system.

The main pathological changes: peripheral nerve damage is mainly caused by different degrees of myelin loss, combined with mild axonal degeneration, small blood vessels of the adventitia have inflammatory changes, vascular wall destruction, lumen narrowing or occlusion, Electron microscopic observation of the catabolic products containing myelin in Schwann cells, axonal disappearance, muscle damage showed inflammatory changes in muscle tissue, muscle atrophy, necrosis and perivascular inflammatory cell infiltration, may be associated with obvious Microangiitis, swelling and degeneration of intravascular vascular endothelial cells, thickening of the basement membrane, endothelial cells can protrude into the lumen, causing stenosis or occlusion of the lumen, and diffuse necrotizing vasculitis and meningoencephalitis can be seen in brain damage.

Sjogren's syndrome nervous system damage prevention

There are no good preventive measures for Sjogren's syndrome. Early diagnosis and early treatment are necessary for patients with SS and neurological damage. For those with genetic factors, genetic counseling, preventive measures include avoiding close relatives, genetic testing of carriers and prenatal Diagnosis and selective abortion, etc.

Sjogren's syndrome neurological damage complications Complications systemic lupus erythematosus dermatomy scleroderma

In addition to the above, SS patients often have dry eyes, dry mouth, dry skin, this disease can also overlap other connective tissue diseases such as rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, scleroderma, etc. It can also be accompanied by Raynaud's phenomenon.

Sjogren's syndrome, nervous system damage symptoms, common symptoms, disturbance of consciousness, sensory disturbance, oral ulcer, dry soles, dry eyes, mental disorders, dysphagia, paralysis, ectopic corneal ulcer

1. Central nervous system damage reported in the literature with SS and central nervous system damage accounted for about 20%, may be focal, multifocal or diffuse brain and / or spinal cord damage, neurological symptoms mostly appear after SS, but a few Can appear before SS, according to the location of the damage, clinical manifestations vary, can be expressed as hemiplegia, aphasia, partial sensory disturbance, hemianopia, focal epilepsy and symptoms of brain stem and cerebellar damage; can also be expressed as Signs of meningeal encephalitis such as disturbance of consciousness, mental disorders, meningeal irritation and abnormal components of cerebrospinal fluid are more common in mental disorders, Parkinson's syndrome, and epilepsy.

2. The symptoms of spinal cord injury are relatively small, which may be manifested as symptoms and signs of chronic progressive myelopathy or acute transverse myelitis. Rare spinal cord subarachnoid hemorrhage, most of which are not abnormal.

3. Peripheral nerve damage SS complicated by peripheral nerve damage is more common, its incidence rate of 30%, can be manifested as symptoms of cranial nerve damage, more common with trigeminal nerve damage, manifested as one or both sides of facial numbness and feeling Decreased, may be accompanied by corneal reflex weakening, corneal ulcer, tongue pain and oral ulcers, in addition, peripheral facial paralysis, oculomotor nerve spasm, olfactory disappearance and pupil diplopia can also occur, cranial nerve damage can also be multiple Sexual and recurrent, spinal nerve damage can be manifested as multiple peripheral neuropathy, multiple mononeuritis, more common in sensory peripheral neuropathy, manifested as limb symmetry glove-like sensation, accompanied by numb acupuncture, lower extremity symptoms It appears earlier and heavier than the upper limbs; or numbness and paresthesia of the damaged nerve distribution area may be associated with motor abnormalities, the electromyogram is denervated, and the motor and sensory nerve conduction velocity is slowed down.

4. Muscle damage symptoms Muscle symptoms can appear before and after SS, mainly manifested as weakness of the proximal muscles of the limbs, muscle weakness, can be accompanied by pronunciation and difficulty in swallowing, EMG is myogenic damage.

5. Other reports have reported that SS can be combined with autonomic nerve damage, manifested as pupillary changes, no sweat and urinary dysfunction, and some with renal tubular acidosis, manifested as hypokalemic periodic paralysis.

Severe syndrome neurological damage examination

1. General examination and other connective tissue diseases, white blood cells can be increased, no specificity.

2. Serological examination of 90% SS patients with polyclonal IgG increased significantly, 85% CIC (+), 70% ANAAs (+), 56% CRP (+), 52% RP (+), normal complement, anti-caries The glandular antibody and SS-A, SS-B, and SS-C antibodies were all elevated, and the SS-A antibody was significantly increased.

3. The number of CSF cells can be increased, mainly due to the increase of monocytes and plasma cells, the protein content is increased, the IgG content is relatively increased, and the chance of oligoclonal bands in CSF protein electrophoresis is higher.

4. EEG can be focal or diffuse slow wave or sharp wave activity.

5. Brain CT or MRI can be seen in areas with limited density reduction.

6. Cerebral angiography sometimes shows changes in cerebral vascular stenosis.

7. Muscle and peripheral nerve biopsy showed inflammatory changes in muscle tissue, muscle atrophy, necrosis and perivascular inflammatory cell infiltration; nerve bundle Schwann cells contain myelin decomposition products, axons disappear.

Diagnosis and diagnosis of nervous system damage in Sjogren's syndrome

diagnosis

According to the patient's medical history, there are dry eyes, dry mouth, dry skin, SS-A and SS-B autoantibodies in the surrounding blood, and symptoms of nervous system damage, if accompanied by other connective tissue diseases such as rheumatoid Arthritis, systemic lupus erythematosus (SLE), etc., should be highly suspected of SS with neurological damage.

Lumbar puncture CSF examination, electromyography and brain CT or MRI should be performed, and muscle and peripheral nerve biopsy should be performed if necessary.

Differential diagnosis

It should be differentiated from multiple sclerosis, various types of meningoencephalitis, muscle disease and peripheral neuropathy.

The material in this site is intended to be of general informational use and is not intended to constitute medical advice, probable diagnosis, or recommended treatments.