Abstract

Interstitial lung disease (ILD) and pulmonary fibrosis comprise a wide array of inflammatory and fibrotic lung diseases which are often confusing to general medicine and pulmonary physicians alike. In addition to the myriad of clinical and radiologic nomenclature used in ILD, histopathologic descriptors may be particularly confusing, and are often extrapolated to radiologic imaging patterns which may further add to the confusion. We propose that rather than focusing on precise histologic findings, focus should be on identifying an accurate etiology of ILD through a comprehensive and detailed medical history. Histopathologic patterns from lung biopsy should not be dismissed, but are often nonspecific, and overall treatment strategy and prognosis are likely to be determined more by the specific etiology of ILD rather than any particular histologic pattern. In this review, we outline a practical approach to common ILDs, highlight important aspects in obtaining an exposure history, clarify terminology and nomenclature, and discuss six common subgroups of ILD likely to be encountered by general medicine physicians in the inpatient or outpatient setting: Smoking-related, hypersensitivity pneumonitis, connective tissue disease-related, occupation-related, medication-induced, and idiopathic pulmonary fibrosis. Accurate diagnosis of these forms of ILD does require supplementing the medical history with results of the physical examination, autoimmune serologic testing, and chest radiographic imaging, but the importance of a comprehensive environmental, avocational, occupational, and medication-use history cannot be overstated and is likely the single most important factor responsible for achieving the best possible outcomes for patients.

Highlights

  • Interstitial lung disease (ILD) and pulmonary fibrosis comprise a wide array of lung diseases which are often confusing to general medicine and pulmonary physicians alike

  • Some examples include cystic forms of ILD, pulmonary eosinophilic syndromes, pulmonary alveolar proteinosis, graft-versus-host disease, and ILD associated with systemic inheritable disorders

  • Post-infectious ILD is often overlooked as a common cause of radiographic or histopathologic findings which suggest an organizing pneumonia (OP) pattern of disease, but we believed this was beyond our scope

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Summary

Introduction

Interstitial lung disease (ILD) and pulmonary fibrosis comprise a wide array of lung diseases which are often confusing to general medicine and pulmonary physicians alike. One of the confusing areas in ILD is the myriad of terms, abbreviations, and acronyms that are applied to imaging patterns, histopathology findings, and integrated clinical diagnoses. Our goals in this review are to highlight the importance of a comprehensive medical history, to provide a practical framework that promotes understanding of major etiologic groups of ILDs, to enhance clarity regarding current nomenclature, J. IILLDD OOvveerrvviieeww IILLDD aanndd ppuullmmoonnaarryy ffiibbrroossiiss aarree aa ggrroouupp ooff lluunngg ddiisseeaasseess wwhhiicchh ccoonnssiisstt ooff aa ccoommbbiinnaattiioonn ooff iinnffllaammmmaattiioonn aanndd ffiibbrroossiiss ooff tthhee lluunngg ppaarreenncchhyymmaa ((FFiigguurree 11)). TThheerreeaarreemmaannyyddiivveerrsseeccaauusseessooffIILLDD,, wwhhiicchh uussuuaallllyyrerseusultltfrofrmoma vaarvieatryieotyf eonfviernovnimroennmtael,natvalo,caavtioocnaatli,ooncaclu, poactciounpaalt,ioornaml,edoircamtieodni-crealtaiotendreexlpaotesdureexsp, oorsuarlteesr,noartiavletleyrnmaatiyverelysumltafyrormesounlteforof mtheonnuemofertohuesnsuymsteemroiucsausytostimemmicunaeutoorimcomnnuencetivore ctiosnsuneecdtiivseeatsisessu(eCdTiDse)a[s1e–s3](.COTDne) [p1a–r3t]ic. Ionf scelliencicteadl iinnfsotarmncaetsi,oonnme paiyecseuopfecrlsiendicealailnl footrhmerastiionnamrraiyvisnugpeartsaecdceuarlaltoethdeiarsgninoasirsr,ivbiuntgmatoasctcoufrtaetne, diniateggnroastiiso,nbouft tmheosmt oefdtiecna,lihnitsetgorraytiionncoofntjhuencmtieodnicwalithhissteorroyloignyc, oimnjuagnicntigonanwdithhissteorloolgoygyfi,nidminaggisnigs aenssdenhtiisatloilnogoyrdfeinr dtoinegsstaibsliesshseanptiraelciisne odridagernotosisesatnadblgisuhidaepthreecriaspeeduitaicgndoesciissioannsd[g2,u6i,d10e,1th1]e.rapeutic decisions [2,6,10,11]

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