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.