Executive Summary Objectives Participants Evidence Validation Key Messages Introduction Rationale for a Change in the Approach to Classification of Idiopathic Interstitial Pneumonias Development of a New Classification of Idiopathic Interstitial Pneumonia Current Classification of IIP New ATS/ERS Classification Principles Guiding the Assessment of Patients with Idiopathic Interstitial Pneumonias The Diagnostic Process Is Dynamic Clinical Evaluation Radiological Evaluation Role of Surgical Lung Biopsy Unclassifiable Interstitial Pneumonia Bronchoalveolar Lavage Fluid Evaluation Idiopathic Pulmonary Fibrosis Clinical Features Radiologic Features Histologic Features IPF: Areas of Uncertainty Nonspecific Interstitial Pneumonia Clinical Features Radiologic Features Histologic Features NSIP: Areas of Uncertainty Cryptogenic Organizing Pneumonia Clinical Features Radiologic Features Histologic Features COP: Areas of Uncertainty Acute Interstitial Pneumonia Clinical Features Radiologic Features Histologic Features AIP: Areas of Uncertainty Respiratory Bronchiolitis-Associated Interstitial Lung Disease Clinical Features Radiologic Features Histologic Features RB-ILD: Areas of Uncertainty Desquamative Interstitial Pneumonia Clinical Features Radiologic Features Histologic Features DIP: Areas of Uncertainty Lymphoid Interstitial Pneumonia Clinical Features Radiologic Features Histologic Features LIP: Areas of Uncertainty References Appendix

American thoracic society/European respiratory society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias

Many acute and chronic lung disorders with variable degrees of pulmonary inflammation and fibrosis are collectively referred to as interstitial lung diseases (ILDs) or diffuse parenchymal lung diseases. Idiopathic pulmonary fibrosis (or cryptogenic fibrosing alveolitis) (IPF or CFA) is one of several idiopathic interstitial pneumonias. IPF is now recognized as a distinct clinical disorder. Despite major accomplishments in our understanding of the pathogenesis of lung fibrosis (l), the diagnosis and management of patients with IPF continues to pose significant challenges (24).

Idiopathic pulmonary fibrosis : Diagnosis and treatment. International consensus statement

Objective Classical dermatomyositis (CDM) patients display the hallmark cutaneous manifestations of dermatomyositis (DM), proximal muscle weakness, and laboratory evidence of myositis. The epidemiology and management of both adult-onset and juvenile-onset CDM has been well characterized. However, the clinical significance of the hallmark inflammatory cutaneous manifestations of DM occurring in individuals who have no clinically significant muscle weakness and normal muscle enzymes for prolonged periods of time (ie, 6 months or longer) has not been clear. The term amyopathic DM (ADM) (synonymous with DM sine myositis ) has been proposed to draw attention to such individuals. A related form of DM, "hypomyopathic DM" [HDM], is the presence of DM skin disease for 6 months or longer in individuals who have no muscle weakness but who are found to have some evidence of muscle inflammation upon testing (muscle enzyme levels, electromyogram, muscle biopsy, muscle magnetic resonance imaging [MRI]). Clinically amyopathic DM (CADM) is a designation that has been proposed for patients having either ADM or HDM. The clinically amyopathic component of this designation was coined to emphasize the fact that the only clinical problem being experienced by these patients at the time of diagnosis is their DM skin disease. Our personal experience suggests that the CADM subphenotype might be more prevalent in adults than has been thought previously. To test this hypothesis and address questions relating to the optimal management and prognosis of such patients, we have systematically reviewed the published literature in this area. Methods We carried out a systematic review of the published literature on adult-onset CADM as defined in Table I through May 1, 2004. Results We identified 291 adult-onset CADM cases (18 years or older) reported from over 19 countries. The average duration of DM skin disease was 3.74 years (range, 6 months [by definition] to > 20 years), and 73% were female. Among 37 patients with HDM who were identified, the average duration of disease was 5.4 years, and none had developed clinically significant weakness at the time of the reports. Thirty-seven of the reported CADM patients developed muscle weakness greater than 6 months after onset of their skin disease (15 months to 6 years). For the sake of this discussion, such patients have been analyzed under the designation of "CADM → CDM." Somewhat surprisingly, 36/291 (13%) of the identified published CADM patients developed interstitial lung disease. Incidental to our review, we also identified 10 published cases of individuals having DM skin disease and interstitial lung disease without muscle weakness, 7 of whom died from interstitial lung disease less than 6 months after onset of their DM skin disease (the term pre-myopathic DM coined by others has been used here to refer to such patients). In addition, an associated internal malignancy was found in 41/291 (14%) of the identified CADM cases. A positive antinuclear antibody was reported in 63% and myositis-specific autoantibodies (eg, Jo-1, Mi-2) in only 3.5% of the reported CADM patients in which such data were available. Conclusions The results of this analysis suggests that the CADM subphenotype is more common than has been thought previously and that such patients may comprise a relatively high proportion of DM patients followed by dermatologists. Some CADM patients also have been observed to develop overt proximal muscle weakness years after onset of their DM skin disease. In addition, CADM patients appear to be at risk of developing the same potentially fatal disease associations/complications for which CDM patients are at risk (eg, interstitial lung disease and internal malignancy). Population-based studies of the epidemiology and optimal management of CADM patients, including efforts to identify risk factors associated with potentially fatal outcomes such as late-onset muscle weakness, interstitial lung disease, and malignancy, are needed. As an incidental finding to this literature review, we also identified a small number of reported cases of often-fatal interstitial lung disease occurring shortly after the onset of DM skin disease (

https://www.jaad.org/article/S0190-9622(05)03345-1/pdf

A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis siné myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies.

This document is an update of the original 1993 statement on community-acquired pneumonia, incorporating new information about bacteriology, patient stratification, diagnostic evaluation, antibiotic therapy, and prevention. The statement includes a summary of the available literature, as well as evidence-based recommendations for patient management, developed by a multidisciplinary group composed of pulmonary, critical care, general internal medicine, and infectious disease specialists. The sections of this document are as follows: an overview of the purpose of our efforts and the methodology used to collect and grade the available data; a review of the likely etiologic pathogens causing community-acquired pneumonia (CAP), including a discussion of drug-resistant Streptococcus pneumoniae (DRSP); a proposed approach to patient stratification for the purpose of predicting the likely etiologic pathogens of different patient populations with CAP; a summary of available and recommended diagnostic studies; suggestions on how to define the need for hospitalization and admission to the intensive care unit (ICU) for patients with CAP; guidelines for antibiotic therapy of CAP, including principles of therapy and specific recommendations for each patient category; an approach to the nonresponding patient, as well as a discussion of when to switch to oral therapy and when to discharge an admitted patient with CAP who is responding to initial therapy; and recommendations for the use of pneumococcal and influenza vaccines.

/pdf/american-thoracic-society-100-years-of-advances-in-the-51j0w5coit.pdf

American Thoracic Society 100 Years of Advances in the Treatment and Science of Respiratory Diseases; 20-25 May 2005; San Diego, California, USA

Nontuberculous mycobacteria (NTM) are environmental microbes that cause a variety of human diseases, particularly chronic lung infections. Despite the fact that NTM are widespread in the environment, relatively few people develop NTM lung disease, suggesting intrinsic vulnerability in some individuals. This paper reviews the evidence that underlying disorders predispose to NTM lung disease, in particular primary conditions that result in bronchiectasis, chronic obstructive pulmonary disease, α-1-antitrypsin anomalies, pneumoconiosis, pulmonary alveolar proteinosis, and frank immunosuppressive states such as that associated with the use of anti-tumor necrosis factor-α biologics, posttransplantation immunosuppression, and HIV infection. Over the past several decades, NTM lung disease has been increasingly identified in postmenopausal women with slender body habitus. Thus we will also review the clinical and experimental evidence which supports the observation that such individuals are predisposed to NTM lung disease.

Underlying host risk factors for nontuberculous mycobacterial lung disease.

Serial changes of cystic air spaces in fibrosing alveolitis: A CT-pathological study

A case of a 41 years old Japanese woman with widely disseminated primary lung cancer (adenocarcinoma) metastasizing to renal cell carcinoma (hypernephroma) is presented together with a brief review of the literature.

Metastasis of cancer to cancer

Gender and underlying diseases affect the frequency of the concurrence of adult polymyositis/dermatomyositis and interstitial pneumonia.

A 39-year-old woman was admitted to our hospital because of dry cough. She had been quite well until 1 month before admission. She had no history of chronic sinusitis. Chest X-ray film showed a thin-walled cavity in the superior segment of the left lower lobe of the lung. Curettage biopsy of the cavity wall revealed granulomatous change. Mycobacterium intracellulare-avium complex was identified on sputum culture. Anti-tuberculous therapy was given and the cavity disappeared in 3 months. Since atypical mycobacterial infection is quite unusual for a healthy woman to contract, we performed high-resolution CT (HRCT) in search for occult abnormalities of the lung. HRCT detected a lot of thin-walled cystic lesions, and bronchography showed that they were consistent with cystic dilatation of relatively large bronchi, which ballooned up during inspiration, and collapsed during expiration. Airways peripheral to these dilatations were also visible, and were quite different from usual cystic bronchiectasis formed by acquired recurrent infections. Her bronchiectasis is most likely congenital in its etiology, and might be considered as an adult case of Williams-Campbell syndrome.

[A probable case of adult Williams-Campbell syndrome incidentally detected by an episode of atypical mycobacterial infection].

Successful closure of bronchopleural fistula by omental pedicle flap in a case of typhoid empyema 43 years after enteric fever

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