[Research progress of serum biomarkers in interstitial lung disease associated with connective tissue disease].
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间质性肺病（ILD）是结缔组织病（CTD）最常见和最严重的并发症之一，极大地影响患者的预后及生存率。早期诊断、准确分类和密切监测对有效管理CTD相关ILD患者至关重要。近年来国内外大量研究表明，CTD相关ILD患者血清生物标志物对疾病诊断、风险分层、预后评估等方面具有重要意义。本文对CTD相关ILD血清生物标志物（自身抗体、肺泡上皮损伤标志物、细胞因子、肿瘤标志物等）的研究进展进行系统综述。.Keywords:
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Pulmonary arterial hypertension (PAH) may complicate diverse connective tissue diseases (CTDs) such as systemic sclerosis (SSc), systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and mixed CTD (MCTD) and is an important cause of morbidity and mortality in this context. From a histological standpoint, the pulmonary vascular lesions in PAH complicating CTD are similar to those observed in idiopathic PAH (IPAH). However, prognosis and responsiveness to therapy are distinctly worse in PAH associated with CTD. PAH is most common in SSc (affecting up to 20% of patients with SSc), and most of the data regarding incidence, clinical features, and therapy of CTD-associated PAH are derived from patients with SSc (scleroderma). Although PAH may involve other CTDs, data are more limited (primarily small, uncontrolled series and case reports). Treatment strategies for PAH associated with CTD are similar to but are distinctly less effective than those for IPAH. Lung transplantation may be offered for a subset of patients with CTD-associated PAH, but results are less favorable than for IPAH due to comorbidities and specific complications associated with CTD (e.g., esophageal involvement, aspiration with SSc).
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Pulmonary arterial hypertension (PAH) is an entity that is known to complicate connective tissue diseases (CTD). PAH in CTD is a very important diagnosis which greatly affects treatment and prognosis. The most commonly affected CTD is scleroderma, although lupus, inflammatory myopathies such as poly and dermatomyositis, and mixed CTD are also associated with PAH. The manifestations of PAH have both similarities and differences when occurring in the setting of CTD as compared with idiopathic PAH. These differences are most notable in scleroderma. In this section we will discuss the features of PAH as they appear in CTDs, and in particular, scleroderma. The focus of this article is an approach to the diagnosis and treatment of PAH in CTD, and how this setting might differ from idiopathic and other forms of PAH.
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In Brief Interstitial lung disease is a common manifestation of connective tissue disease, and connective tissue disease-associated interstitial lung disease (CTD-ILD) is associated with significant morbidity and mortality. The treatment of CTD-ILD is often complex and challenging. This review focuses on the management of CTD-ILD. Interstitial lung disease is a common pulmonary manifestation of the connective tissue diseases and is associated with significant morbidity and mortality. Connective tissue disease-associated interstitial lung diseases are also among the most difficult to treat. This review focuses up the management of connective tissue disease-associated interstitial lung disease.
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Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial lung disease (ILD) which is associated with CTD is referred to as CTD-ILD. Patients diagnosed with ILD should be thoroughly examined for the co-occurrence of CTD, since the treatment procedures and prognosis of CTD-ILD are vary from those of idiopathic interstitial pneumonia. The representative types of CTD which may accompany ILD include rheumatoid arthritis, systemic sclerosis (SSc), Sjögren's syndrome, mixed CTD, idiopathic inflammatory myopathies, and systemic lupus erythematous. Of these, ILD most frequently co-exists with SSc. If an ILD is observed in the chest, high resolution computed tomography and specific diagnostic criteria for any type of CTD are met, then a diagnosis of CTD-ILD is made. It is challenging to conduct a properly designed randomized study on CTD-ILD, due to low incidence. Therefore, CTD-ILD treatment approach is yet to been established in absence of randomized controlled clinical trials, with the exception of SSc-ILD. When a patient is presented with acute CTD-ILD or if symptoms occur due to progression of the disease, steroid and immunosuppressive therapy are generally considered.
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Background
Connective tissue disease (CTD) is a type of autoimmune disease that affects many organs and tissues. The most common manifestations in the lungs include interstitial lung disease, pulmonary arterial hypertension (PAH), pulmonary infarction, and pleuritis. Most CTD-PAH patients without symptoms or with mild PAH are overlooked by doctors and therefore cannot be diagnosed or treated early. In this study, we retrospectively analysed the clinical manifestations, laboratory features and therapeutic regimens of 111 CTD-PAH patients selected from 2584 CTD patients to improve our understanding of this disease.Objectives
This study aimed to estimate the incidence of pulmonary arterial hypertension (PAH) in connective tissue disease (CTD) patients, assess the relationship between the patients' clinical manifestations and the type of PAH, and discuss the effects of different diseases and treatments on prognosis.Methods
A retrospective evaluation of 111 CTD-PAH patients from 2584 CTD patients was conducted. Clinical data and experimental indexes were collected from the patients at baseline and after 1,2,3,5 years of treatment to investigate the risk factors of PAH and to assess whether different treatments or primary diseases would make a difference in the prognosis.Results
CTD-PAH was most prevalent in SSc (18.86%) and MCTD (12.00%) patients compared with other CTD patients (P<0.01). Patients with mild or moderate PAH exhibited a much better response to treatment than patients with severe PAH. SSc patients had worse clinical manifestations and haemodynamic parameters than SLE patients at the baseline and after treatment. The mortality rate in CTD-PAH patients was significantly lower than in CTD patients without PAH (0.85% vs 6.31%, P <0.05). The 5-year survival rate for targeted PAH therapy group has become significantly higher than non-targeted PAH therapy group (0.91% vs 0.71%, P=0.028).Conclusions
Raynaud's phenomenon, anti-U1RNP(+), and ACA-IgG(+) are suggestive of a higher risk of PAH in patients with CTD. The treatment outcomes in patients with severe PAH or patients with SSc and PAH were both poor. Targeted therapy can improve the 5-year survival rate of CTD-PAH patients.References
Galie N, Torbicki A, Barst R, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J 2004;25:2243–78. British Cardiac Society Guidelines and Medical Practice Committee. Recommendations on the management of pulmonary hypertension in clinical practice. Heart, 2001,86 Suppl 1:11-13. Koh ET, Lee P, Gladman D, Abu-Shakram M. Pulmonary hypertensionin systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 1996; Williams MH, Das C, Handler CE, Akram MR, Davar J, Denton CP, Smith CJ, Black CM, Coghlan JG. Systemic sclerosis associated pulmonary. Chung SM, Lee CK, Lee EY, Yoo B, Lee SD, Moon HB. Clinical aspects of pulmonary hypertension in patients with systemic lupus erythematosus and in patients with idiopathic pulmonary arterial hypertension. Clin Rheumatol 2006;25:866–872. Horn CA. Pulmonary hypertension and autoimmune disease. Chest, 1993, 104: 279-282.Disclosure of Interest
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Connective tissue disease (CTD) are closely related to liver abnormality. CTD can affect the liver causing various degrees of liver injury, coexist with other liver diseases, especially autoimmune liver disease (ALD). Medications for CTD can also lead to liver injury or reactivate the hepatitis B virus. CTD patients can also be positive for ALD-related autoantibodies without corresponding manifestation; and vis versa. The diagnosis and differential diagnosis should be made on integrating clinical presentation, laboratory, imaging, and histological studies, not solely relying on autoantibody positivity.结缔组织病（CTD）与肝脏关系密切。CTD可以累及肝脏并导致不同程度的肝损伤，也可以合并其他肝脏疾病尤其是自身免疫性肝脏疾病（ALD）。此外，治疗CTD的药物也可以引起肝脏损伤或激活乙型肝炎病毒。需要注意的是，ALD可出现CTD相关的自身抗体阳性，而无CTD的其他表现；反之亦然。因此，其诊断和鉴别诊断需综合分析患者的临床表现、实验室检查、影像学检查，甚至组织病理学检查，不能仅凭相应的自身抗体阳性就下结论。.
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Frequency and clinical manifestations of respiratory disease in systemic connective tissue diseases (CTD) differ significantly including clinical course and outcomes. Pulmonary abnormalities are seen in 20% to 95% of patients with CTD ranging from subtle to life-threatening disease and could be fatal. Commonly, CTD-related interstitial lung disease (ILD) is crucial for prognosis. Interstitial pneumonias are the most frequent variant of respiratory disease in patients with CTD. CTD-related ILDs comprise 15% to 25% of all ILDs. Usually, respiratory disease develops in symptomatic CTD, but in some cases, ILD can be the first appearance of CTD which precedes systemic symptoms that could significantly impede early diagnosis. Therefore, diagnostic workup in ILD should consider extrapulmonary manifestations of CTD. Moreover, an intrinsic part of diagnostic algorithm should be specific laboratory assessment including measurement of autoantibodies. Combination of specific antibodies and clinical features provides higher probability of CTD diagnosis.
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Interstitial lung disease (ILD) is a common manifestation of connective tissue disease (CTD) and is often associated with significant morbidity and mortality. The evaluation of ILD in patients with CTD is complex because of the heterogeneity of the CTD spectrum, the various patterns and degrees of severity of ILD encountered, and because ILD can be identified at any point in time in these patients. A thorough - and optimally multidisciplinary - evaluation is needed when CTD patients develop ILD or when evaluating ILD patients for the presence of occult CTD. Determining whether ILD is associated with a preexisting CTD requires the exclusion of alternative etiologies, and thorough assessments of the clinical features of both the CTD and ILD. The detection of occult CTD in patients with presumed idiopathic interstitial pneumonia requires careful integration of clinical, serologic, and thoracic imaging and histopathologic features.
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