Once this organ is involved, is the risk of death significantly increased in patients with Sjögren's syndrome?

*Only for medical professionals to read the latest information on Sjögren's syndrome pulmonary lesions! Sjögren's syndrome (SS) is a diffuse autoimmune disease characterized by invasion of exocrine glands such as lacrimal and salivary glands, with a high degree of lymphocyte infiltration and specific autoantibodies, often involving the respiratory and circulatory systems (16.
54%), and the blood system (16.
54%).
13.
33%), joint muscles (7.
17%), kidneys (3.
65%) and other organ systems[1]
.

Primary SS (pSS) refers to SS without other connective tissue diseases
.

The incidence of pulmonary lesions in SS patients is 9% to 75% [2].
The main symptoms are dry cough, dyspnea, dry nose, epistaxis, and disturbance of smell and taste
.

Moreover, with the prolongation of the disease course, the respiratory symptoms continued to increase, and the 10-year mortality rate increased by 4 times compared with that before the disease (17% vs.
4.
5%)
.

It can be seen that reducing lung damage is the key to SS management
.

At the annual meeting of the Rheumatology and Immunology Branch of the Chinese Medical Doctor Association in 2021, Professor Xu Dong from the Department of Rheumatology and Immunology, Peking Union Medical College Hospital gave an in-depth interpretation of the pulmonary involvement of SS, and introduced in detail the interstitial pulmonary disease (ILD) and pulmonary hypertension ( The latest progress in the epidemiology, risk factors and domestic diagnosis and treatment of PAH) has updated the cognition of SS management in the medical field
.

pSS-ILD: The most common pulmonary involvement ILD is the most common complication of SS, with a prevalence of 13.
6% in SS patients.
), lymphatic interstitial pneumonia (LIP), and organizing pneumonia (OP)
.

Professor Xu Dong said that hyperglobulinemia, increased rheumatoid factor (RF) titers, elderly males, smoking, and long disease duration are risk factors for pulmonary involvement in SS, and are also prognostic risk factors for death
.

However, the correlation between ANA titer, anti-SSA antibody and pulmonary involvement is still controversial
.

In addition to advanced age and male sex, the independent risk factors for ILD were increased erythrocyte sedimentation rate (ESR) and positive anti-Ro-52 antibody
.

A national multi-center cohort study found that 46.
5% of SS patients had ILD as the first manifestation, and the interval from the onset of pSS to ILD was 2.
0 months.
The main symptoms were shortness of breath after exercise (41.
1%), followed by sputum and dry cough.
Pulmonary crackles were audible on physical examination of the lungs (23.
6%)
.

The study also found that ANA can be detected in 88.
9% of patients, anti-SSA antibody can be detected in 80.
3%, and the rest include anti-Ro-52 antibody (77.
2%), ACA antibody (66.
7%) and anti-SSB antibody (34.
4%), etc.

.

Professor Xu Dong emphasized that high-resolution CT (HRCT) is an important auxiliary test for the diagnosis of ILD
.

In ILD, ground-glass opacities (50%) and grid opacities (36.
8%) are often seen on HRCT, with occasional stretch bronchiectasis and honeycomb lungs, and subpleural consolidation is rare
.

HRCT can also differentiate NSIP, UIP, LIP, and OP (Figure 1), and the results show that the most common imaging type is NSIP
.

In addition, lung function is also an essential auxiliary test
.

Figure 1 Different types of pSS-ILD The treatment for pSS-ILD, Professor Xu Dong shared the treatment plan applied in the above research, commonly used drugs include glucocorticoid, hydroxychloroquine, cyclophosphamide, tripterygium wilfordii, acetylcysteine, Less commonly used are mycophenolate mofetil, azathioprine, cyclosporine, tacrolimus, and pirfenidone.
Doctors need to choose treatment drugs individually according to the patient's condition
.

pSS-PAH: It is important to maintain low-risk stratification SS can cause changes in pulmonary vascular structure or function, leading to PAH, with a prevalence of 3.
8% in SS patients
.

The consequences of PAH are severe and can develop into right heart failure and even death
.

A multicenter case-control cohort study in China found that pSS patients with younger age at onset, positive anti-SSB and anti-RNP antibodies, and positive corneal staining were more likely to have PAH, and corneal staining was an independent risk factor for pSS combined with PAH [3]
.

Combined PAH also has a negative impact on the prognosis of pSS
.

Data from the National Rheumatology Data Center (CRDC) showed that the 1-year, 3-year, and 5-year survival rates of pSS patients were 99.
3%, 98.
5%, and 97.
7%, respectively, while the 1-year, 3-year, and 5-year survival rates of pSS-PAH patients The rates were 94%, 88.
8%, and 79%, respectively (Figure 2), indicating that PAH increased the mortality of pSS
.

Figure 2 Results of CRDC data Interestingly, the prognosis of pSS-PAH patients with different risk stratification was also "stratified" accordingly
.

In 2015, the European Society of Cardiology/European Society of Radiology (ESC/ESR) issued guidelines, recommending that PAH be risk stratified according to the following indicators: ① WHO functional class; ② 6-minute walking distance; ③ plasma level of NT-proBNP or BNP; ④ hemodynamics Physical indicators [right atrial pressure, cardiac index, mixed venous oxygen saturation (SvO2)]
.

Each item is scored based on the measurement results, and the higher the score, the higher the risk level
.

The study showed that among the low-risk, intermediate-risk, and high-risk pSS-PAH subgroups, the high-risk group had the lowest 1-, 3-, and 5-year survival rates, and the low-risk group had the highest 1-, 3-, and 5-year survival rates (Fig.
3), to prove that the above risk indicators are the specific reasons for the increased mortality of pSS caused by PAH
.

Figure 3 Prognosis of pSS-PAH patients with different risk stratification Professor Xu Dong suggested that pSS patients with younger onset age and positive anti-SSB and anti-RNP antibodies should be routinely screened for PAH
.

Patients diagnosed with pSS-PAH should actively improve cardiopulmonary function, maintain a non-injury state, and especially maintain low risk stratification to improve prognosis
.

In addition to ILD and PAH, pSS pulmonary involvement can also manifest as bronchial wall thickening, mosaic sign, lymphoma, and bullae
.

In the pulmonary assessment of pSS, the first thing to ask is whether SS patients have pulmonary symptoms such as shortness of breath, expectoration, and dry cough after exercise
.

If no abnormality is found on examination, other diseases need to be considered (Figure 4)
.

Figure 4 Pulmonary assessment of pSS According to the "pSS diagnosis and treatment guidelines", SS lung involvement should follow the following treatment principles: 1.
HRCT lung lesions <10%, and no respiratory symptoms, lung diffusing capacity of carbon monoxide (DLCO) % predicted value For patients >65%, it is recommended to monitor closely and evaluate every 6 months or so; 2.
For patients with severe disease and rapid progression, oral or intravenous glucocorticoids can be used, and immunosuppressants can be selected from cyclophosphamide, Mycophenolate, etc.
; 3.
The anti-fibrotic drugs pirfenidone and nintedanib for the treatment of idiopathic pulmonary fibrosis (IPF) have yet to be further confirmed their efficacy on SS complicated with pulmonary fibrosis; 4.
Local inhaled glucocorticoids and β2 adrenergic receptor agonists (such as albuterol) can be used for patients with bronchial lesions; 5.
Acetylcysteine ​​can be used as adjuvant therapy
.

Conclusion The incidence of pulmonary lesions in SS patients is 9%-75%.
ILD is the most common, followed by PAH, which increases the risk of death in SS patients
.

Advanced age, male sex, elevated ESR, and anti-Ro-52 antibody positivity are risk factors for pSS-ILD, which also have adverse effects on prognosis
.

The pSS patients with younger onset age, positive anti-SSB and anti-RNP antibodies, and positive corneal staining were more likely to develop PAH, and the higher the PAH risk stratification, the worse the prognosis
.

Pulmonary function and HRCT are important means of evaluating pulmonary lesions in SS, and medical staff should carry out individualized treatment according to the patient's condition and examination results
.

References: [1] Roca, F.
, et al Autoimmun Rev, 2017.
16(1): 48-54[2] Palm O, et al.
Rheumatology, (Oxford) 2013;52:173-9[3] Wang JY, et al.
Eur Respir J 2020; 56: 1902157