Complications of Sjögren's syndrome

Some people look very hydrated, which is what we often call a watery look, while some people look dry, as if they are extremely dehydrated. Many people often feel dry mouth and tongue, which may be due to the weather. The air is drier in winter, which makes people feel dry mouth. Of course, it is also related to the region. Places with less precipitation will naturally be drier. So when you feel dry mouth and tongue, could it be that you have Sjögren's syndrome? Let's learn about Sjögren's syndrome and its complications

Clinical manifestations

The onset of this disease is often insidious and the clinical manifestations are diverse.

1. Local manifestations

(1) Xerostomia

Due to salivary gland lesions, salivary mucin deficiency causes the following common symptoms: ① Most patients complain of dry mouth. In severe cases, the oral mucosa, teeth and tongue become sticky, so the patient needs to drink water frequently when speaking, and solid food must be taken with water or liquid food. ② Rampant dental caries is one of the characteristics of this disease. About 50% of patients develop multiple uncontrolled caries, which are manifested by the gradual darkening of the teeth, followed by small pieces falling off, and ultimately leaving only residual roots. ③ In adult mumps, 50% of patients present with intermittent alternating parotid swelling and pain, involving one or both sides. Most of them can disappear on their own in about 10 days, but sometimes the swelling persists. A few people have submandibular gland enlargement, while sublingual gland enlargement is less common. ④ The tongue manifests as tongue pain, dry and cracked tongue surface, and atrophied and smooth tongue papillae. ⑤Ulcers or secondary infections occur in the oral mucosa.

(2) Keratoconjunctivitis sicca

This is due to the decrease in mucin secretion by the lacrimal glands, which causes symptoms such as dry eyes, foreign body sensation, and less tears. In severe cases, the patient may cry without tears. Some patients have recurrent purulent infections of the eyelid margin, conjunctivitis, keratitis, etc.

(3) Others

Exocrine glands in superficial areas such as the nose, hard palate, trachea and its branches, gastrointestinal mucosa, and vaginal mucosa can all be affected, causing their secretion to decrease and resulting in corresponding symptoms.

2. System performance

In addition to dry mouth and eyes, patients may also experience systemic symptoms such as fatigue and low fever. About 2/3 of patients experience systemic damage.

(1) Skin

Allergic purpuric rash may occur, more commonly on the lower limbs, and is red papules the size of rice grains with clear borders that do not fade when pressed and appear in batches. Each episode lasts about 10 days and will fade on its own, leaving behind brown pigmentation.

(2) Joint pain is common, and damage to the joint structure is usually not present.

(3) Kidney: About half of the patients have renal damage, mainly affecting the distal renal tubules, and renal tubular acidosis may occur. A small number of patients develop more obvious glomerular damage, with clinical manifestations including massive proteinuria, hypoalbuminemia, and even renal insufficiency.

(4) Lungs

Most patients have no respiratory symptoms. Mildly affected people experience a dry cough, while severe cases experience shortness of breath. The main pathology of the lungs is interstitial lesions, and a small number of patients develop pulmonary hypertension. Patients with pulmonary fibrosis and severe pulmonary hypertension have a poor prognosis.

(5) Digestive system

Non-specific symptoms such as atrophic gastritis, decreased gastric acid, and indigestion may occur, and patients may have liver damage.

(6) Neurological disease: A few cases involve the nervous system. Peripheral nerve damage is the most common.

(7) Blood system

This disease may cause a decrease in white blood cell count or (and) thrombocytopenia. Severe thrombocytopenia may cause bleeding. The incidence of lymphoma in this disease is much higher than that in the normal population.

diagnosis

The currently recognized diagnostic criteria are the 2002 International Classification (Diagnosis) criteria for Sjögren's Syndrome, which are as follows:

Table 1 Items of the classification criteria for Sjögren's syndrome

Ⅰ. One or more of the three oral symptoms.

1. Daily dry mouth lasts for more than 3 months;

2. Recurrent or persistent swelling of the parotid gland in adulthood;

3. Use water to help swallow dry food.

Ⅱ. One or more of the three eye symptoms

1. Unbearable dry eyes lasting for more than 3 months;

2. Repeated sand in the eyes or sanding sensation;

3. Artificial tears need to be used 3 times or more a day.

III. Ocular signs

Any one or more of the following tests are positive

1. Schirmer I test (+);

2. Corneal staining (+).

IV. Histological examination

Pathology of labial gland shows lymphocyte foci

Ⅴ. Damage to salivary glands

Any one or more of the following tests are positive

1. Salivary flow rate (+);

2. Parotid gland angiography (+);

3. Salivary gland isotope examination (+).

VI. Autoantibodies

Anti-SSA or anti-SSB (+) (double diffusion method)

Table 2 Specific classification of the above items

1. Primary Sjögren's syndrome can be diagnosed if there are two of the following conditions:

a. Meet 4 or more of the items in Table 1, but must include item IV (histological examination) and/or item VI (autoantibodies);

b. Any three of the four items III, IV, V, and VI are positive.

2. Patients with secondary Sjögren's syndrome have underlying diseases (such as any connective tissue disease) and meet any one of items Ⅰ and Ⅱ in Table 1, and any two of items Ⅲ, Ⅳ, and Ⅴ.

3. Patients with a history of radiotherapy to the neck, head and face, hepatitis C virus infection, AIDS, lymphoma, sarcoidosis, GVH disease, and the use of anti-acetylcholine drugs (such as atropine, scopolamine, propantheline bromide, belladonna, etc.) must be excluded.