结节病易误诊,据王洪武等~([1])收集国内18篇关于此病误诊的文献,误诊率高达63.2%,当然有误诊就会有误治,如孙永昌等~([2])报道26例结节病在影像学检查诊断的第一印象中拟诊结核5例,其中就有2例完成规范的抗结核治疗,为此应引起临床对本病诊治的重视。

Sarcoidosis

Pulmonary alveolar proteinosis in adults: pathophysiology and clinical approach.

Compared with the extensive data on left‐sided infective endocarditis (IE), there is much less published information on the features and management of right‐sided IE. Right‐sided IE accoun...

Right-Sided Infective Endocarditis 2020: Challenges and Updates in Diagnosis and Treatment.

Purpose of review The formation of noncaseating granuloma is a hallmark of pulmonary sarcoidosis. This review summarizes recent progress made to explain the cellular dynamics within the granuloma structure that may considerably differ between the two clinically distinct variants, that is, acute and chronic sarcoidosis. Recent findings Compelling evidence exists that in acute but not chronic sarcoidosis CD4 T lymphocytes specifically recognizing the auto-antigen vimentin on human leukocyte antigen-DR3 molecules accumulate in sarcoid granuloma. These so-called TH17.1 cells produce high amounts of the TH17-related cytokines interleukin-17 (IL-17) and IL-22 in addition to interferon-γ. Moreover, regulatory T cells from patients with acute sarcoidosis are ICOS, providing a mechanistic link to the comparably high concentration of IL-10 exclusively found in the airways of these patients. Next to obvious differences in T effector cell and Treg subsets, alveolar macrophages harbor a functional mitochondrial system in acute sarcoidosis patients, while this system is impaired in patients with chronic disease. Summary We provide a comprehensive update on the cellular components and their functional implications in sarcoid granuloma formation, with special emphasis on the specific characteristics of granuloma in acute versus chronic sarcoidosis. Moreover, the specific antigens thought to be involved in both forms of the disease are discussed.

Mechanism of granuloma formation in sarcoidosis

Pulmonary alveolar proteinosis

Thorax is the common place to develop Castleman disease (CD), but there is no systemic clinical analysis for intrathoracic CD. We conducted a retrospective analysis of 48 intrathoracic CD patients with definite pathological diagnosis who were hospitalized between 1992 and 2012 in a Chinese tertiary referral hospital. The study included 16 cases with unicentric CD (UCD) and 32 cases with multicentric CD (MCD). UCD were younger than MCD (30.5y vs 41.6ys, P < 0.05). MCD were more symptomatic (50% vs 96.9%, P < 0.001) and sicker than UCD, including more fever, hepatomegaly and/or splenomegaly and hypoalbuminemia. All of UCD showed solitary mass in various sites and two of them were complicated by small pleural effusion. In the MCD group, their chest CT showed obvious lymphadenopathy in the hilum and/or mediastinum (100%), diffuse parenchymal lung shadows (43.75%), pleural effusion (40.6%), mass in the mediastinum (6.25%) or hilum (3.12%) and bronchiolitis obliterans (BO) (3.12%). Besides LIP-like images, multiple nodules of different size and sites, patchy, ground-glass opacities and consolidation were showed in their chest CT. Surgery were arranged for all UCD for diagnosis and treatment and all were alive. In MCD group, superficial lymph nodes biopsies (21 cases), surgery biopsy (9 cases) and CT-guided percutaneous lung biopsy (2 cases) were performed. Hyaline vascular (HV) variant were more common in the UCD group (75% vs 37.5%, P < 0.05). In MCD group, 28 cases were prescribed with chemotherapy, one refused to receive therapy and the rest three were arranged for regular follow-up. Among MCD, 18 cases was improved, 7 cases was stable, 4 cases lost follow-up and 3 cases died. Intrathoracic MCD was more common than UCD in our hospital. MCD was older, more symptomic and sicker than UCD. HV variant were more common in UCD. All of UCD showed mass in various intrathoracic locations and surgery resection was performed for all and all were alive. Mass, pleural effusion, BO and diffuse pulmonary shadows, including LIP-like images, multiple nodules of different size and sites, patchy, GGO and consolidations were showed in our MCD. Most of MCD cases were arranged with chemotherapy and their prognosis were worse than UCD’s.

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Clinical spectrum of intrathoracic Castleman disease: a retrospective analysis of 48 cases in a single Chinese hospital

Objectives
Pulmonary sarcoidosis is an immune-mediated disease, and some patients can be effectively treated with corticosteroids. However, nearly half of all sarcoidosis patients relapse after corticosteroid withdrawal. Different subsets of CD4+ helper T cells participate in the immunopathogenesis of sarcoidosis. Thus, the aims of our study were to investigate whether the circulating subsets of CD4+ helper T cells were associated with sarcoidosis relapse and with its remission after retreatment. Additionally, we identified a useful biomarker for predicting the relapse and remission of sarcoidosis patients.

/pdf/the-circulating-treg-th17-cell-ratio-is-correlated-with-3l7a19k3f9.pdf

The Circulating Treg/Th17 Cell Ratio Is Correlated with Relapse and Treatment Response in Pulmonary Sarcoidosis Patients after Corticosteroid Withdrawal

Cardiac septic pulmonary embolism: A retrospective analysis of 20 cases in a Chinese population.

Background:Pulmonary alveolar proteinosis (PAP) is a rare lung disease,the most common type of which is autoimmune PAP.The gold standard therapy for PAP is whole lung lavage (WLL).Few studies have reported the optimal technique with which to evaluate the response to WLL.In this study,we aimed to identify parameters with which to assess the need for repeat WLL during a long-term 8-year follow-up.Methods:We conducted a retrospective analysis of 120 patients with autoimmune PAP with 80 of whom underwent WLL.Physiologic,serologic,and radiologic features of the patients were analyzed during an 8-year follow-up after the first WLL treatment.Results:Of the 40 patients without any intervention,39 patients either achieved remission or remained stable and only one died of pulmonary infection.Of the 56 patients who underwent WLL for 1 time,55 remained free from a second WLL and 1 patient died of cancer.Twenty-four required additional treatments after their first WLL.The baseline PaO2 (P =0.000),PA-aO2 (P =0.000),shunt fraction rate (P =0.00 1),percent of predicted normal diffusing capacity of the lung for carbon monoxide (DLCO％Pred) (P =0.016),6-min walk test (P =0.013),carcinoembryonic antigen (CEA) (P =0.007),and neuron-specific enolase (NSE) (P =0.003) showed significant differences among the three groups.The need for a second WLL was significantly associated with PaO2 (P=0.000),CEA (P =0.050),the 6-minute walk test (P =0.026),and DLCO％Pred (P =0.041).The DLCO％Pred on admission with a cut-offvalue of42.1％ (P =0.001) may help to distinguish whether patients with PAP require a second WLL.Conclusions:WLL is the optimal treatment method for PAP and provides remarkable improvements for affected patients.The DLCO％Pred on admission with a cut-off value of 42.1％ may distinguish whether patients with PAP require a second WLL.

Whole Lung Lavage Treatment of Chinese Patients with Autoimmune Pulmonary Alveolar Proteinosis: A Retrospective Long-term Follow-up Study

OBJECTIVES Few studies have investigated the prognostic factors for idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD) across different clinical/serological phenotypes. METHODS We conducted a retrospective analysis of patients diagnosed with IIM between January 2012 and December 2017. RESULTS Of the 760 IIM cases registered, 679 adult cases were included in this study. ILD was present in 508 cases, and the presence of ILD in the clinically amyopathic DM, DM and PM groups was 92.7, 73.6 and 55.1%, respectively (P   0.05). The prevalence of ILD in the melanoma differentiation-associated protein-5 (MDA-5)+-IIM group was higher than that in MDA-5--IIM group (97.8 vs 72.1%, P   0.05), although both were higher than that in PM group (13.2%, P = 0.01 and P   0.05), and both were lower than that in MDA5+ASA--IM-ILD group (33.7%, P < 0.05). CONCLUSION The prevalence of ILD in IIM and the prognosis of IIM-ILD patients may vary depending on the statuses of the ASA and MDA-5 antibodies.

Shan Li

Papers

Clinical spectrum of intrathoracic Castleman disease: a retrospective analysis of 48 cases in a single Chinese hospital

The Circulating Treg/Th17 Cell Ratio Is Correlated with Relapse and Treatment Response in Pulmonary Sarcoidosis Patients after Corticosteroid Withdrawal

Cardiac septic pulmonary embolism: A retrospective analysis of 20 cases in a Chinese population.

Whole Lung Lavage Treatment of Chinese Patients with Autoimmune Pulmonary Alveolar Proteinosis: A Retrospective Long-term Follow-up Study

Prognosis of adult idiopathic inflammatory myopathy-associated interstitial lung disease: a retrospective study of 679 adult cases