Current opinion in pulmonary medicine
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A subset of patients with interstitial lung diseases (ILDs), such as rheumatoid arthritis (RA)-associated ILD and chronic hypersensitivity pneumonitis, will experience a disease course similar to patients with idiopathic pulmonary fibrosis (IPF). They also often have a usual interstitial pneumonia (UIP) pattern of fibrosis. Although the standard of care for patients with RA-ILD and chronic hypersensitivity pneumonitis is immunosuppression, the optimal treatment for patients with progressive disease and a UIP pattern remains unknown. ⋯ IPF shares several clinical, genetic and biological features with other ILDs exhibiting the UIP pattern. The optimal pharmacologic management of these patients remains uncertain. Several ongoing trials are evaluating the efficacy of antifibrotic medications in these other diagnoses and may change how we approach ILD treatment.
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Usual interstitial pneumonia (UIP) pattern is the histologic marker of idiopathic pulmonary fibrosis (IPF), but usefulness of ancillary histologic findings may discriminate idiopathic from secondary UIP. ⋯ High-resolution computed tomography identifies a 'definite' UIP pattern in about half of cases, failing to recognize UIP in the absence of honeycombing or in limited disease. Although radiologic criteria for UIP need redefinition to improve their diagnostic yield, histologic features of UIP did not significantly change from the 1960s but continue to represent a major diagnostic tool, particularly in challenging interstitial lung diseases. A careful recognition of some histologic ancillary findings in UIP (e.g., cellular/follicular bronchiolitis with germinal centers, chronic pleuritis, interstitial granulomas/giant cells, bridging fibrosis) may be helpful in supporting secondary forms (e.g., connective tissue disease, chronic hypersensitivity pneumonia) from IPF. Cryobiopsy and awake-biopsy are promising approaches to obtain representative lung tissue preventing conventional surgical lung biopsy. Genomic techniques have recently demonstrated good-to-high sensitivity and specificity to disclose UIP pattern starting from RNA obtained in transbronchial biopsy, possibly replacing and/or flanking soon traditional histology.
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Advanced sarcoidosis is an important cause of morbidity and mortality in sarcoidosis. Over the past few years, several studies have been published clarifying the prevalence and severity of this condition. ⋯ Advanced sarcoidosis is associated with significant morbidity and some mortality. Up to a quarter of all sarcoidosis patients have one or more forms of advanced disease. These patients require closer monitoring and often multiples treatments.
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Benign pleural effusions are common and usually managed by treating the underlying disease process. In some patients, these effusions may be refractory to medical management. Indwelling pleural catheters, used extensively for malignant pleural effusions, are increasingly used in benign pleural effusions not responding to medical therapy. This review focuses on current data regarding their safety and effectiveness in effusions due to congestive heart failure, hepatic hydrothorax, end-stage renal disease, and chylothorax. ⋯ Indwelling pleural catheters are effective at symptom palliation and have pleurodesis rates comparable to that seen in malignant pleural effusions. However, given the paucity of evidence and low quality of available data, prospective and comparative studies evaluating safety and efficacy in these specific patient populations are needed.
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Malignant pleural effusion is a common cause of breathlessness and signifies advanced disease. Common options for definitive pleural intervention include insertion of an indwelling pleural catheter (IPC) or talc pleurodesis. ⋯ In patients where an ambulatory treatment pathway is preferred, the increased chance of pleurodesis with talc administration via IPC can result in a faster time to device removal and may be associated with better quality of life and symptom scores.