Small problems such as dry mouth and dry eyes may also be a sign of a serious illness?

It is only for medical professionals to read for reference.
Be wary of this rheumatism! Case brief introduction The patient, female, 59 years old, was admitted to the hospital for "chest tightness and cough for 1 year after the activity, aggravated sputum and hoarseness for 3 weeks"
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History of present illness: The patient developed chest tightness and cough symptoms after activity in May 2020, and increased coughing when lying supine at night.
There was no obvious cause of coughing and increased sputum production a month before, accompanied by yellow and sticky sputum, hoarse voice and dry throat.
At the local health center, he was given treatments such as heat-clearing and detoxifying, cough relieving, and phlegm removal.
Two days later, the hoarseness improved, and the symptoms of cough, sputum and chest tightness did not improve
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At the beginning of May, he went to a local hospital.
On May 3, 2021, a chest CT scan revealed two interstitial changes in the lungs
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He was treated with ceftazidime + levofloxacin for anti-infection and methylprednisolone anti-inflammatory treatment for 3 days without significant improvement .

For further diagnosis and treatment to the outpatient clinic of our hospital, the outpatient department is admitted to our department with "interstitial lung disease"
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Past history, personal history, and family history are nothing special
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The patient complained of obvious symptoms of dry mouth and eyes
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Admission examination: T: 36.
6℃, P: 76 beats/min, R: 18 beats/min, BP: 122/74mmHg, SpO2: 99%
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Consciousness, no cyanosis of the lips, Velcro rales can be heard in both lower lungs, no clubbing, regular heart rhythm, no pathological murmur in the auscultation area of ​​each valve, flat and soft abdomen, no tenderness, rebound pain, no lower limbs Edema
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Preliminary diagnosis: Interstitial pneumonia was admitted to the hospital.
Preliminary treatment: Moxifloxacin was given for anti-infection, acetylcysteine ​​to reduce phlegm and symptomatic supportive treatment
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After treatment: 2021-05-08 Laboratory examination: blood routine: WBC 6.
74×109/L, NEUT% 56%, PLT 280×109/L
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Blood biochemistry: ALT 15U/L, AST 18U/L, TBIL 3.
2↓μmol/L, ALB 42.
79g/L, K+4.
3mmol/L, Na+138mmol/L, UREA 3.
95mmol/L, Cr 47.
57umol/L
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Blood gas analysis: pH 7.
43, PO2 12.
3KPa, PCO2 5.
77KPa, BE 4.
2↑mmol/L
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Tumor markers: CA19-9 60.
84U/ml, CA-50 42.
1U/ml, and there are no obvious abnormalities
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Autoantibodies: antinuclear antibody positive (1:160), SS-A positive, RO-52 positive, and the rest negative
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Humoral immunity: IgA 4.
46↑g/L, IgG 17.
6↑g/L, IgM 1.
11g/L, complement C3 1.
02g/L, complement C4 0.
17g/L
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There were no obvious abnormalities in blood coagulation function, hepatitis B virus hepatitis markers, the first three items of blood transfusion, erythrocyte sedimentation rate, G test, GM test, and sputum acid-fast bacilli smear
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On May 10, 2021, anti-inflammatory treatment with methylprednisolone 40 mg qd was added
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Ophthalmology consultation on May 11, 2021: Schirmer test (+), diagnosis of binocular dry eye
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Bronchoscopic lung biopsy (TBLB) was performed
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Labial gland biopsy will be performed on 2021-05-13
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2021-05-13 Chest CT examination: grid-like shadows and interstitial changes can be seen on the near dorsal sides of both lungs
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There are a few patches and small nodules in the remaining lung
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Figure 1 Lung CT2021-05-17 right lower lobe dorsal segment TBLB lung biopsy pathology: submitted for small focal mucosal chronic inflammation, acid-fast staining (-), PAS staining (-)
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Pathology of labial gland biopsy on May 18, 2021: Lymphocyte infiltration (1 focus, >200) around the duct, and individual lymphocyte infiltration in the lobules, combined with clinical conditions, can be consistent with the pathological changes of Sjogren’s syndrome
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2021-05-21 discharge diagnosis: Sjogren’s syndrome with interstitial pneumonia
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After discharge from the hospital, he continued to receive oral treatment with methylprednisolone tablets 20 mg qd and hydroxychloroquine tablets 200 mg qd
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On 2021-06-21, the outpatient review CT showed similar to the previous one
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Figure 2 Lung CT★Experts commented on Tang Haicheng, director of the Department of Respiratory and Critical Care Medicine, Shanghai Public Health Clinical Center, Fudan University.
Some patients with interstitial pneumonia have abnormalities in the autoimmune system, called "interstitial pneumonia with autoimmune characteristics" (IPAF)", some individuals who were initially diagnosed with IPAF may develop a certain type of connective tissue disease over time
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IPAF diagnostic criteria: 1.
The presence of interstitial pneumonia (confirmed by HRCT or lung biopsy); 2.
Excluding other known causes; 3.
It is not yet certain that it meets a certain connective tissue disease diagnosis; 4.
There are at least the following 3 Two of the characteristics: a.
Clinical manifestations: including cracks in the skin of the distal fingers (such as "artisan hands"); skin ulcers on the distal fingertips; inflammatory arthritis or multi-joint morning stiffness ≥60 minutes; palm or fingertips Vasodilatation; Raynaud's phenomenon; unexplained finger edema and unexplained fixed rash on the back of the finger (Gottrons sign)
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b.
Serological manifestations: ANA positive> 1:320 (diffuse, spot, homogeneous type) or ANA nucleolar type (any titer) or ANA centromere type (any titer); RF> 2 times the upper limit of normal; Positive for one of the following antibodies: anti-CCP, anti-dsDNA, anti-Ro (SSA), anti-La (SSB), anti-RNP, anti-Sm, anti-SCL-70, anti-tRNA synthetase (such as Jo-1, PL- 7, PL-12, others such as EJ, OJ, KS, Zo, tRS), anti-PM-Scl and anti-MDA-5 and the like
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c.
Morphological manifestations: Radiologically, high-resolution CT suggests the following types: non-specific interstitial pneumonia; organizing pneumonia; non-specific interstitial pneumonia and overlapping organizing pneumonia; lymphocytic interstitial pneumonia
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The patient has symptoms of dry mouth and dry eyes.
Serological tests for antinuclear antibodies, SS-A and RO-52 are positive, and elevated IgG all indicate that the patient is interstitial pneumonia with autoimmune characteristics.
It is necessary to further clarify whether there is any clinically.
Connective tissue disease-related interstitial lung disease (CTD-ILD), primary Sjogren Syndrome (pSS) should be considered first
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The diagnostic criteria for pSS are more complicated, including inclusion criteria and exclusion criteria
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1.
Inclusion criteria: Those who have at least one of the symptoms of dry eyes or dry mouth, that is, at least one of the following is positive: (1) Feeling unbearable dry eyes every day for more than 3 months; (2) Repeated grit in the eyes Feeling; (3) Need to use artificial tears 3 or more times a day; (4) Feel dry mouth every day for more than 3 months; (5) Swallow dry foods need frequent drinking water to help
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Or at least one system-positive suspected pSS in the pSS Disease Activity Index (ESSDAI) questionnaire of the European Union Against Rheumatism (EULAR)
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2.
Exclusion criteria.
Patients with the following diseases should be excluded because they may have overlapping clinical manifestations or interfere with the diagnostic test results: (1) history of head and neck radiotherapy; (2) active hepatitis C virus infection; (3) AIDS; (4) Sarcoidosis; (5) Amyloidosis; (6) Graft versus host disease; (7) IgG4-related diseases
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Those who meet all the inclusion criteria and exclude the exclusion criteria, and the European Union Against Rheumatism-American College of Rheumatology (EULAR-ACR) score ≥ 4 points can be diagnosed as pSS
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According to the above diagnostic criteria, this patient meets the inclusion criteria, excludes related diseases, and scores at least 6 points, so it can be diagnosed as primary Sjogren’s syndrome
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Lung disease can be diagnosed as interstitial lung disease caused by primary Sjogren’s syndrome
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2016 EULAR-ACR Sjogren’s Syndrome Diagnosis Score Sheet: Table 1 2016 EULAR-ACR Sjogren’s Syndrome Diagnosis Score Sheet Discovered by Wu Qingguo, Director of the Department of Respiratory and Critical Care Medicine, Shanghai Public Health Clinical Center Affiliated to Fudan University, in a study of domestic interstitial pneumonia Connective tissue disease-related interstitial pneumonia accounts for 67% of the total population, and the mortality rate is higher than that of patients with simple connective tissue disease, especially those with pulmonary hypertension.
The clinical difference is large.
In mild cases, there may be no clinical symptoms, but in severe cases.
Patients can develop respiratory failure at the beginning and rapidly progress to death, such as anti-MDA5 antibody-positive amyopathy dermatomyositis-related interstitial lung disease, which is closely related to rapidly progressive interstitial lung disease
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Clinical manifestations often include cough, shortness of breath, joint swelling and pain, morning stiffness, dry mouth, rash, muscle pain, Raynaud’s phenomenon, etc.
The most characteristic sign is velcro rales in the lungs
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Patients with different types of connective tissue diseases can have different combinations of positive antibodies
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Among the serological markers, KL-6 is worthy of attention.
Studies have shown that the level of KL-6 in patients with connective tissue disease with interstitial pneumonia is significantly higher than that in patients with connective tissue disease or healthy people without interstitial pneumonia
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Among autoantibodies related to connective tissue disease, special attention should be paid to patients with positive anti-synthetase antibodies.
Such patients have a high probability of eventually developing interstitial pneumonia
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Lung biopsy is the gold standard for the diagnosis of interstitial pneumonia, especially bronchoscopy frozen lung biopsy (TBCB)
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This is extremely important for the pathological classification of interstitial pneumonia, but because it is an invasive examination, there is the possibility of inducing deterioration of the disease, so clinical implementation is difficult
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We performed TBLB for this patient.
Compared with TBCB, the tissue samples are too small to affect the pathological diagnosis.
This shows the importance of TBCB from another perspective
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In terms of treatment, connective tissue disease-related interstitial pneumonia is divided into general treatment, induction treatment, maintenance treatment and other treatments
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General treatment: including cardiopulmonary rehabilitation training, smokers quit smoking, systemic sclerosis with gastroesophageal reflux, antacid therapy, patients with pulmonary hypertension can be appropriately given to reduce pulmonary hypertension, but the ultimate benefit is limited
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In the course of treatment, we need to closely observe whether the patient has high-risk factors for infection and the basis of infection.
Once found, we must actively give anti-infective treatment
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Induction therapy: Glucocorticoid is the most important drug for induction therapy of connective tissue disease-related interstitial pneumonia
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For most patients with severe or rapidly progressing connective tissue disease-related interstitial pneumonia, cyclophosphamide combined with glucocorticoids is the basic treatment option, but it is necessary to pay attention to the bone marrow suppression caused by cyclophosphamide, increase the risk of infection and increase the incidence of tumors Wait for the situation
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Maintenance treatment: After induction, maintenance treatment can be started.
The drugs include azathioprine, mycophenolate mofetil, and tacrolimus
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Generally speaking, azathioprine as a maintenance treatment after induction therapy can effectively maintain stable lung function
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Studies have shown that mycophenolate mofetil and tacrolimus can effectively reduce the dose of oral glucocorticoids in patients
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Other treatments: including the biological agent rituximab, may be effective for some patients with connective tissue disease-related interstitial pneumonia who are severely or rapidly progressing, but need to pay attention to whether there are respiratory infections
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Another important issue is that the current anti-fibrosis treatments, including pirfenidone and nintedanib, have no clear opinions on the timing and duration of medication
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For patients with particularly severe interstitial pneumonia, lung transplantation can be considered
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