The Results of a Three Year Analysis on Sarcoidosis Patients Registered in the Regis Electronic Registry

AbstractIntroduction. Registries are necessary particularly for rare diseases. The REGIS registry was developed to improve scientific research in the field of interstitial lung diseases (ILDs).Material and Method. We analysed 144 patients with interstitial lung diseases enrolled during a three year period, selected the sarcoidosis cases and analysed the data with Excel 2007 Software.Results. A number of 26 sarcoidosis patients were included (18.06% out of the total), of which 46.15% were female, with an average age of 41.34±9.84 years; 23.08% were smokers. 88.46% pf patients had mediastinum pulmonary involvement: stage II in 17 patients, stage III in 5 patients and stage IV in one patient; only 3 patients had extra thoracic involvement. The medical investigations performed were: chest X-ray (96.15%), thoracic HRCT (96.15%) that identified hilar adenopathy (96%), nodules (68%), with peribronchovascular localization (44%); spirometry (96.15%) that revealed an average CV of 94.99±23.07% out of the predicted value, DLCO 78.41±19.39% out of the predicted value; bronchoalveolar lavage (38.46%); serum angiotensin-converting enzyme (73.08%) and pulmonary or lymph node biopsy (80.77%). The patients that needed treatment (46.15%) were administered oral corticosteroids (10 patients), acetylcysteine (1 patient) and pirfenidone (1 patient).Conclusions. The number of patients enrolled is low as a result of the voluntary reporting, especially in severe cases and with access to all investigations needed for diagnosing.

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The Role of Transthoracic Ultrasound in the Study of Interstitial Lung Diseases: High-Resolution Computed Tomography Versus Ultrasound Patterns: Our Preliminary Experience

Diagnostics ◽

10.3390/diagnostics11030439 ◽

2021 ◽

Vol 11 (3) ◽

pp. 439

Author(s):

Donato Lacedonia ◽

Giulia Scioscia ◽

Angelamaria Giardinelli ◽

Carla Maria Irene Quarato ◽

Ennio Vincenzo Sassani ◽

...

Keyword(s):

Computed Tomography ◽

High Resolution ◽

Lung Diseases ◽

Interstitial Lung Diseases ◽

High Resolution Computed Tomography ◽

Pleural Line ◽

Complementary Role ◽

Number Of Patients ◽

Significant Difference ◽

Transthoracic Ultrasound

Transthoracic ultrasound (TUS) is a readily available imaging tool that can provide a quick real-time evaluation. The aim of this preliminary study was to establish a complementary role for this imaging method in the approach of interstitial lung diseases (ILDs). TUS examination was performed in 43 consecutive patients with pulmonary fibrosis and TUS findings were compared with the corresponding high-resolution computed tomography (HRCT) scans. All patients showed a thickened hyperechoic pleural line, despite no difference between dominant HRCT patterns (ground glass, honeycombing, mixed pattern) being recorded (p > 0.05). However, pleural lines’ thickening showed a significant difference between different HRCT degree of fibrosis (p < 0.001) and a negative correlation with functional parameters. The presence of >3 B-lines and subpleural nodules was also assessed in a large number of patients, although they did not demonstrate any particular association with a specific HRCT finding or fibrotic degree. Results allow us to suggest a complementary role for TUS in facilitating an early diagnosis of ILD or helping to detect a possible disease progression or eventual complications during routine clinical practice (with pleural line measurements and subpleural nodules), although HRCT remains the gold standard in the definition of ILD pattern, disease extent and follow-up.

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Chest X-Ray Refresher

The Brigham Intensive Review of Internal Medicine ◽

10.1093/med/9780199358274.003.0037 ◽

2014 ◽

pp. 379-389

Author(s):

Christopher H. Fanta

Keyword(s):

Lung Diseases ◽

Interstitial Lung Diseases ◽

X Rays ◽

X Ray ◽

Obstructive Lung Diseases ◽

Chest X Ray

This Chest X-Ray Refresher is organized as a game. For each of the three topics to be discussed, we offer four chest x-rays and four clinical histories. The order of each set is random. The exercise asks that you consider the clues in the history and the findings on chest x-ray to match the history with the x-ray. In many instances, the combination will suggest a diagnosis or a limited differential of diagnostic possibilities. The three topics to be discussed are hemoptysis, chronic interstitial lung diseases, and obstructive lung diseases.

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Dynamics in diagnoses and pharmacotherapy before and after diagnosing idiopathic pulmonary fibrosis

ERJ Open Research ◽

10.1183/23120541.00479-2020 ◽

2020 ◽

Vol 6 (4) ◽

pp. 00479-2020

Author(s):

Jesper Rømhild Davidsen ◽

Lars Christian Lund ◽

Christian B. Laursen ◽

Jesper Hallas ◽

Daniel Pilsgaard Henriksen

Keyword(s):

Drug Use ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Diseases ◽

Interstitial Lung Diseases ◽

Opium Alkaloids ◽

Before And After ◽

Oral Corticosteroids ◽

Drug Treatments ◽

Respiratory Drugs

BackgroundIdiopathic pulmonary fibrosis (IPF) is a well-characterised interstitial lung disease. Typically, IPF diagnosis is delayed due to nonspecific symptoms, but can also be delayed due to treatment attempts on false indication or due to treatment targeting common comorbidities. This observational study aimed to assess the dynamics in the medication and diagnosis patterns in the period before and after an IPF diagnosis.MethodsWe identified all Danish patients with IPF between 2002 and 2017. We evaluated new and ongoing drug treatments and incident diagnoses 36 months before and 12 months after an IPF diagnosis by use of Danish nationwide registries. To aid interpretation, 10 random controls were recruited for each case.ResultsA total of 650 IPF patients were identified (median age 73 years (interquartile range 65–78), 70.3% males). Prior to the IPF diagnosis, the most prevalent diagnoses were dyspnoea and non-IPF interstitial lung diseases. For drug use, IPF patients had higher initiation rates for antibiotics, oral corticosteroids and mucolytics. In terms of drug volume, IPF patients used more respiratory drugs, antibiotics, immunosuppressants, corticosteroids, proton pump inhibitors, benzodiazepines and opium alkaloids within the 6 months preceding their IPF diagnosis, compared to the controls. Overall drug use decreased after an IPF diagnosis, mainly due to a reduced glucocorticoid and cardiovascular drug use.ConclusionAmong IPF patients, an increased drug use was observed for diagnoses with symptoms overlapping those of IPF, particularly this was observed during the last 6 months before an IPF diagnosis. This emphasises the need for an increased IPF awareness.

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Validation of the artificial intelligence software to detect chronic fibrosing interstitial lung diseases in chest X-ray

10.1183/13993003.congress-2021.oa1211 ◽

2021 ◽

Author(s):

Hirotaka Nishikiori ◽

Kenichi Hirota ◽

Tomohiro Suzuki ◽

Yuso Takagi ◽

Seiwa Honda ◽

...

Keyword(s):

Artificial Intelligence ◽

Lung Diseases ◽

Interstitial Lung Diseases ◽

X Ray ◽

Chest X Ray

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Extracellular Heat Shock Proteins as Therapeutic Targets and Biomarkers in Fibrosing Interstitial Lung Diseases

International Journal of Molecular Sciences ◽

10.3390/ijms22179316 ◽

2021 ◽

Vol 22 (17) ◽

pp. 9316

Author(s):

Julie Tanguy ◽

Lenny Pommerolle ◽

Carmen Garrido ◽

Martin Kolb ◽

Philippe Bonniaud ◽

...

Keyword(s):

Heat Shock ◽

Heat Shock Proteins ◽

Lung Diseases ◽

Drug Exposure ◽

Stress Proteins ◽

Therapeutic Targets ◽

Interstitial Lung Diseases ◽

Vast Number ◽

Number Of Patients ◽

Shock Proteins

Interstitial lung diseases (ILDs) include a large number of diseases and causes with variable outcomes often associated with progressive fibrosis. Although each of the individual fibrosing ILDs are rare, collectively, they affect a considerable number of patients, representing a significant burden of disease. Idiopathic pulmonary fibrosis (IPF) is the typical chronic fibrosing ILD associated with progressive decline in lung. Other fibrosing ILDs are often associated with connective tissues diseases, including rheumatoid arthritis-ILD (RA-ILD) and systemic sclerosis-associated ILD (SSc-ILD), or environmental/drug exposure. Given the vast number of progressive fibrosing ILDs and the disparities in clinical patterns and disease features, the course of these diseases is heterogeneous and cannot accurately be predicted for an individual patient. As a consequence, the discovery of novel biomarkers for these types of diseases is a major clinical challenge. Heat shock proteins (HSPs) are molecular chaperons that have been extensively described to be involved in fibrogenesis. Their extracellular forms (eHSPs) have been recently and successfully used as therapeutic targets or circulating biomarkers in cancer. The current review will describe the role of eHSPs in fibrosing ILDs, highlighting the importance of these particular stress proteins to develop new therapeutic strategies and discover potential biomarkers in these diseases.

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Interstitial Pneumonia in Patients with Idhiopatic Thrombocytopenic Purpura.

Blood ◽

10.1182/blood.v104.11.3953.3953 ◽

2004 ◽

Vol 104 (11) ◽

pp. 3953-3953

Author(s):

Vincenzo Fontana ◽

Elio Donna ◽

Pamela Dudkiewicz ◽

Gabriella Lander ◽

Yeon S. Ahn

Keyword(s):

Acute Lung Injury ◽

Pulmonary Fibrosis ◽

Lung Injury ◽

Interstitial Pneumonia ◽

Lung Diseases ◽

Interstitial Lung Diseases ◽

Mild Symptom ◽

Thrombocytopenic Purpura ◽

X Ray ◽

Chest X Ray

Abstract INTRODUCTION: Idhiopatic thrombocytopenic purpura (ITP) is an autoimmune disease characterized by a premature destruction of platelets by macrophage, especially in the spleen. However in some cases, platelet sequestration and destruction may occur in other organs. Chromium labeled platelet sequestration study revealed that liver or precordial area are prominent sites of sequestration in some cases, suggesting that the lung might be the site in certain cases. Some cases of interstitial pneumonia are associated with immunologic injury to the lung and seen in patients with some autoimmune diseases, infections, drugs and transfusion related acute lung injury (TRALI) in which transfusions of platelets and blood products induce acute lung injury due to sequestration of platelets and neutrophils in lungs, sometimes leading to ARDS. We describe here an unusual association between ITP and interstitial pneumonia, suggesting that a lung injury similar to TRALI is involved in acute and recurrent ITP. METHODS: We have identified patients with ITP who developed interstitial pneumonia during the course of ITP. We reviewed their charts and analyzed their clinical courses of ITP and interstitial lung diseases. Laboratory tests and chest X ray or CAT scans were reviewed. The laboratory study included CBC, platelets and platelets activation was measured by PMP (platelet microparticles), expression of CD62p flowcytometrically. RESULTS: We have identified 6 patients with ITP who developed interstitial pneumonia during the course of ITP. In two of six, interstitial pneumonia was detected at the presentation of acute ITP. ITP was severe with platelet counts less than 10.000. Interstitial pneumonia was discovered incidentally by chest X ray and confirmed by CAT scans. A mild symptom of dyspnea was detected in careful examination. One underwent lung biopsy which showed findings consistent with brochiolitis obliterans organizing pneumonia. Repeated CAT scans in 1–3 months revealed marked improvement but residual interstitial infiltrates still persisted. Four others had a long standing chronic ITP with clinical courses characterized by frequent relapses in spite of surgical and medical therapy. Four of six patients had splenectomy. Interstitial lung diseases were detected at the time of a severe relapse with platelet counts of less than 20.000. One patient underwent chromium labeled platelet sequestration study which revealed rapid sequestration of platelets in the lung. Interstitial infiltrates improved following improvement of ITP but two progressed to interstitial pulmonary fibrosis. CD62P measured by flowcytometry was very high in all 3 patients tested, indicating persisting platelet activation in this clinical setting. SUMMARY: We report interstitial pneumonia developing in 6 patients with ITP. Clinically all were asymptomatic and detection of interstitial pneumonia was incidental radiology finding. A mild symptom of exertional dyspnea was present in careful investigation. Chest X ray or CT scans showed nonspecific interstitial infiltrates and showed an overall improvement within months but residual infiltrates persisted. Two progressed to pulmonary fibrosis. We suggest that platelets are sequestered and destroyed in the lung in some patients with ITP, to generate cytokines and lipid mediators that lead to a nonspecific interstitial lung disease.

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Transbronchial cryobiopsy: an effective tool in the diagnosis of lymphoproliferative disorders of the lung

ERJ Open Research ◽

10.1183/23120541.00260-2019 ◽

2020 ◽

Vol 6 (3) ◽

pp. 00260-2019

Author(s):

Rita Bianchi ◽

Alessandra Dubini ◽

Silvia Asioli ◽

Claudia Ravaglia ◽

Sara Tomassetti ◽

...

Keyword(s):

Lung Diseases ◽

Pulmonary Involvement ◽

Interstitial Lung Diseases ◽

Lymphoproliferative Disorders ◽

Definitive Diagnosis ◽

Severe Bleeding ◽

Surgical Biopsy ◽

Molecular Tests ◽

Transbronchial Lung Cryobiopsy ◽

Ancillary Studies

IntroductionMalignant lymphoproliferative disorders are rarely observed in the lung and, considering their clinical and radiological heterogeneity, diagnosis is often difficult and may require invasive methods. Transbronchial cryobiopsy has been confirmed as a new tool in the diagnosis of interstitial lung diseases, given its fewer risks and costs compared to surgical approach. This study is aimed at assessing the effectiveness of cryobiopsy in the diagnosis of lymphoproliferative disorders.Materials and methodsAmong 970 consecutive cryobiopsies, performed between January 2011 and June 2018 at Morgagni Hospital of Forlì, Italy, 13 cases of lymphoproliferative disorders were collected.ResultsIn 12 out of 13 cases a precise pathological diagnosis could be reached with the support of immunohistochemistry (IHC) and molecular ancillary studies. In the only case in which cryobiopsy did not lead to a definitive diagnosis, the subsequent surgical biopsy also did not help to clarify the diagnosis. Severe bleeding or pneumothorax did not occur in any case. On average, five biopsies were obtained per case, with a mean total area of 1161 mm2, and only 5 out of 65 specimens were inadequate for diagnosis. Instant freezing did not produce tissue artefacts nor did it affect IHC and molecular tests. In all cases the amount of available tissue was sufficient for all ancillary studies.ConclusionsTransbronchial lung cryobiopsy is safe and effective for diagnosis in patients with suspected pulmonary involvement by lymphoproliferative disorders and it should therefore be considered a valid alternative to surgical biopsy in such cases.

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TO STUDY THE SIGNIFICANCE OF HRCT OVER CHEST X- RAY IN THE DIAGNOSIS OF INTERSTITIAL LUNG DISEASES

Journal of Evolution of Medical and Dental Sciences ◽

10.14260/jemds/2019/21 ◽

2019 ◽

Vol 8 (2) ◽

pp. 94-98

Author(s):

Manoj Kumar Agrawal ◽

Amit Kumar ◽

Rajesh Agrawal ◽

Rishi Rana

Keyword(s):

Lung Diseases ◽

Interstitial Lung Diseases ◽

X Ray ◽

Chest X Ray

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Interstitial lung diseases with progressive pulmonary fibrosis: pathogenetic features and approaches to therapy

Medical Council ◽

10.21518/2079-701x-2020-17-99-106 ◽

2020 ◽

pp. 99-106

Author(s):

N. A. Kuzubova ◽

O. N. Titova ◽

D. B. Skliarova

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Interstitial Pneumonia ◽

Lung Diseases ◽

Usual Interstitial Pneumonia ◽

Connective Tissue Diseases ◽

Immunosuppressive Drugs ◽

Interstitial Lung Diseases ◽

Fibrotic Process ◽

Number Of Patients

A number of patients with interstitial lung diseases (ILD) of various etiologies, including hypersensitive pneumonitis, diffuse connective tissue diseases (rheumatoid arthritis, systemic scleroderma, dermatomyositis), sarcoidosis, idiopathic non-specific interstitial pneumonia (NSIP) and unclassified ILD develop rapid deterioration of lung ventilation function due to the progression of fibrotic changes, accompanied by a decrease in physical performance and quality of life. It is proposed to distinguish a progressive fibrotic phenotype from those with similar pathogenetic mechanisms, radiologic pattern, clinical course, and prognosis. The progressive course of the fibrotic process is assessed by reducing the forced vital capacity of the lungs (FVC), increasing the severity of signs of pulmonary fibrosis according to computed tomography (CT) and worsening respiratory symptoms. There are several risk factors for the progression of ILD, such as male gender, older age, lower initial pulmonary function, and radiological or pathological picture of usual interstitial pneumonia (UIP). Currently, the role of antifibrotic drugs in the treatment of this pathology is being actively studied. Previously, the common approach was to use this group of drugs in patients with idiopathic pulmonary fibrosis (IPF) and immunosuppressive drugs in patients with other fibrotic subtypes of IL. However, the results of clinical studies have shown a favorable response to antifibrotic therapy for a wider range of fibrotic ILD, manifested in a decrease in the annual rate of FVC reduction. And in 2020, the use of the first anti-fibrotic drug was approved for the treatment of patients with advanced pulmonary fibrosis, NOT related to idiopathic pulmonary fibrosis (IPF).

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Dermatomyositis and Granulomatous Myopathy Associated with Sarcoidosis

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/s0317167100028195 ◽

1988 ◽

Vol 15 (4) ◽

pp. 426-429 ◽

Cited By ~ 5

Author(s):

Jeffrey H. Lipton ◽

B. Daniel McLeod ◽

A. Keith W. Brownell

Keyword(s):

Lymph Node ◽

Mediastinal Lymph Node ◽

Lymph Node Biopsy ◽

X Ray ◽

Node Biopsy ◽

Hilar Adenopathy ◽

Chest X Ray

ABSTRACT:A previously healthy, 21-year-old female presented with acute dermatomyositis. Chest X-ray revealed bilateral hilar adenopathy, and mediastinal lymph node biopsy demonstrated noncaseating granulomata compatible with sarcoidosis. The patient improved spontaneously. The significance of bilateral hilar adenopathy in association with dermatomyositis and implications for management are discussed.

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