*For medical professionals for reference
Can a disease explain all the symptoms of Aunt Zhu?
Writing 丨 Jin Di Wang Yuwei
I will share it with you todayThis case is clearly diagnosed with a clear diagnosis of rheumatism.After more than ten years of diagnosis of hybrid connective tissue disease (MCTD), patients gradually began to intolerance for activities, and even dyspnea in daily activities, improved the diagnosisIt is a rare lung disease.Have you encountered similar cases?
Case Introduction
The patient was 46 -year -old, and was in 1998.Diagnosis is MCTD, clinical manifestations include muscleitis, Renault phenomena, limb sclerosis and other manifestations, and anti -U1RNP antibody positive.Personal history and family history are not special, and the history of smoking is denied.
At the end of 2005, patients began to develop dry symptoms. The Schirmer test was positive and diagnosed with secondary drying syndrome.
In 2008, there was no obvious abnormality in the X -ray of the chest (Figure 1A).
Figure 1 A, B, C comparison of the X-ray comparison of chest X-ray in 2008, 2015 and 2016
In December 2015, patients were admitted to the hospital for "fever and cough".
chest sketch: In addition to the actual change of the left left leaf, the bilateral permeable wire -shaped and mesh infiltration shadows are scattered in small cystic lesions (Figure 1B).
Sophomic microorganisms are prompting golden bacteria infection.After considering the left lower left pulmonary pneumonia, the patient was treated with ammonia gyanicillin and Schuban, and the patients fever and cough symptoms quickly relieved.However, labor difficulty has not improved significantly.
In January 2016, the review of the thoracic tablet showed that the lower left lung leaf changed significantly, but the bilateral lungs continued to have cystic lesions and mesh infiltration (Figure 1c).
Perfect indicators such as laboratory testing (see Table 1) for comprehensive assessment, no evidence such as anemia, thyroid dysfunction, or other autoimmune diseases.
Table 1. Laboratory testing indicators
Multiple leaflet centers and scattered cysts are scattered, especially with bilateral middle and lower pulmonary fields (Figure 2A-D).
Figure 2A-D, E-H December 2015 and July 2016 patient chest CT image comparison
Pulmonary function test: moderate obstructive ventilation dysfunction and severe diffuse dysfunction.
Patients further improve the thoracoscopic examination. The surgeon conducts a biopsy at the middle of the right lung, and takes a lung tissue with a volume of about 6cm × 4cm × 0.8cm.During the operation, a multi -cystic nodular lesions on the right lung surface can be seen under the thoracoscopy (Figure 3).
Figure 3 Figure 3 See you under the thoracoscopy. You can see the right lung surface multi-cystic nodular lesions
The most significant tissue manifestations of lung biopsy specimens are densely infiltrated by multiple lesions, and multiple small and medium -sized lymphocytes are mixed with pulp cells and tissue cells.CD5, CD10 and BCL-2 immunohistochemical dyeing is negative.They form filter foaming in the interstitial of near and far away from the fine -sized trachea. The filter is mainly composed of CD20 positive B cells and CD3 positive T cells peripherals.
Multiple (PCR) combined heterosexual dual-chain analysis shows the polyxia of these lymphocytes.Microbial culture of lung biopsy specimens did not find the growth of any bacteria, branches or fungi.Non -specific characteristics of interstitial pneumonia were found.It can be seen that the spare cellulose -like vasculitis is only a small lesion.
Combined with the diagnosis of "lymphocytic interstitial pneumonia (LIP)" in combination with the clinical, lung imaging and tissue pathological manifestations of patients.Patients have a clear history of MCTD in the past.The treatment is used to apply glucocorticoids, starting from the dose of 40mg/day of metrophic nylon, gradually decreases to 10mg/day maintenance.
Follow -up follow -up: Patients check the lung function regularly. At first, it was prompted that the lung volume parameters were improved compared with the previous, and then showed a relatively stable trend (Table 2).After 7 months, followed the chest CT, and the mesh and cystic lesions did not progress significantly (Figure 2e-H).
Case Small Case
In the year, more than 10 years after the diagnosis of MCTD, patients had difficulty in performing labor breathing difficulties. After improving the chest CT, lung biopsy and other examinations, secondary LIP, glucocorticoid therapy was effective.This case is published in the Respiratory Medicine Case Reports magazine. It is the first report of LIP as the main lung manifestation of MCTD, providing important reference value for our clinical work.
Next, we hit the iron while hot, learn about the related knowledge of MCTD and LIP ~
LIP was first described by Liebow and Carrington in 1969of.Unlike lymphoma, it is usually considered a benign pulmonary lymph hyperplasia, and has dense dopon B and T cells in the interstitial.Typical symptoms include dyspnea, cough and fatigue.The early clinical and X -ray manifestations of LIP lack specificity. High -resolution CT can find bilateral diffuse glass shadows, small leaf centers, thickening and scattered cystic lesions, pulmonary hypertension and honeycomb signs are advanced performanceEssence
The original LIP is rare. Once the LIP diagnosis is determined, it is necessary to further find the cause of the lip.Common causes are autoimmune diseases, human immune defect infections, EB virus infections, nodule diseases, and mucosal -related lymphoma.Patients with LIP on the disease are often diagnosed with a sexual cough and dyspnea, and then found diseases such as dry syndrome.The relationship between LIP and dry syndrome, systemic lupus erythematosus, rheumatoid arthritis, Hashimoto thyroiditis, primary bile liver cirrhosis, malignant anemia, and chronic active hepatitis have been repeatedly reported.
MCTD is a systemic autoimmune disease with complicated clinical manifestations, including Renault phenomena, arthritis, limb sclerosis, esophageal dysfunction, kidney disease and inflammatory muscleitis, etc.EssenceAnti-U1 RNP antibody is highly related to MCTD. The clinical characteristics of this patient are in line with the classification standards of Alarcón-Segovia and Kahn. MCTD diagnosis is reliable.Common CT manifestations of MCTD lungs include grinding glass -like shadow, subtidal nodules, thickening of leaflet intervals and fibrosis.The most common MCTD -related interstitial pulmonary disease is non -specific inter -pneumonia.
Disposal experience
The lung is its own itselfCommonly involved organs of immune diseases are mostly manifested as lung quality and airway.For patients who have previously diagnosed autoimmune diseases, they cannot ignore the rare lung lesions.This example is MCTD correlation LIP. At present, it still needs to study its actual prevalence, clarify its pathogenesis and standardize treatment strategies.
Conclusion
References:
[1] chin-wei, kung-chao, et al.Lymphocytic Internal Pneumonia in a Patient with Mixed Connective Tissue Disease-A Case Report. [J] .Respiratory Medicine Case Reports, 2018.
[2] He Quanye. Persistent lung fiberClinical diagnosis of clinical diagnosis [J]. Clinical lung magazine, 2018,023 (001): 1-4.
[3] Yu Guohua, Su Chunfang, Zhang Yong, etc.. Thin-wall cyst cavity is a secondary lymphocytic interstitial pneumonia with imaging characteristics. Two cases of interstitial interstitial pneumonia [J]. International breathing magazine, 2015,035 (024): 1860-1863.
Source: Rheumatology and Immune Channel in the medical world
Editor: Taro
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