Interstitial pneumonia with autoimmune features ipaf. Mycophenolate therapy in interstitial pneumonia with autoimmune features. Interstitial pneumonia with autoimmune features and platypnea. Download specialty imaging hrct of the lung 2e pdf. Giacomo grasselli1,beatrice vergnano 2,maria rosa pozzi3,vittoria sala 2,gabriele dandrea4, vittorio scaravilli1,marco mantero5,alberto pesci6,7and antonio pesenti1,5 abstract.
Differentiating connective tissue diseaseassociated interstitial lung disease from idiopathic pulmonary fibrosis jonathan h. Patients with idiopathic interstitial pneumonia may display evidence of autoimmunity without meeting criteria for a defined connective tissue disease. Approximately onethird of patients with ild have autoimmune manifestations not classifiable as a ctd, designated as interstitial pneumonia with autoimmune features ipaf. The following case from our clinic supports our position and should resonate among those who routinely evaluate patients with an interstitial lung disease ild. All interstitial lung diseases affect the interstitium, a part of your lungs. Moreover, the diagnostic approach can be limited by the severity of the disease, which may not allow invasive exams. Clinical characteristics of interstitial pneumonia with. This new classification system incorporates not only clinical and serological manifestations of ctd, but also morphological features suggestive of a ctd encountered on highresolution computed tomography hrct, surgical lung biopsy slb and pulmonary function testing pfts. Nonspecific interstitial pneumonia nsip is a rare disorder that affects the tissue that surrounds and separates the tiny air sacs of the lungs. Nonspecific interstitial pneumonia cleveland clinic. A particular subset of interstitial pneumonia, associated to one or more clinical and serological features, suggesting a possible underlying autoimmune disorder, has been described and recently named interstitial pneumonia with autoimmune features ipaf. Interstitial pneumonia with autoimmune features annals of the. In 2015, ats ers working group proposed the term interstitial pneumonia with autoimmune features ipaf as a possible spectrum of ctdild along with its provisional criteria eur respir j 2015. Sep 24, 2019 interstitial pneumonia with autoimmune features confers an increased risk of developing a systemic autoimmune rheumatic disease.
Many patients with an idiopathic interstitial pneumonia iip have clinical features that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease ctd. Carter, reflects the many recent changes in hrct diagnostic interpretation. This discussion highlights the diagnosis of and treatment approach for interstitial pneumonia with autoimmune features ipaf. Sep 27, 2019 a significant proportion of patients with interstitial lung disease ild manifest autoimmune features, but do not fulfill the diagnostic criteria for a definite connective tissue disease ctd. Giacomo grasselli1,beatrice vergnano 2,maria rosa pozzi3,vittoria sala 2,gabriele dandrea4. These air sacs, called the alveoli, are where the exchange of oxygen and carbon dioxide takes place between the lungs and the bloodstream. Recently the term interstitial pneumonia with autoimmune features ipaf has been proposed to identify patients with interstitial lung disease and autoimmune characteristics, not fulfilling the criteria for specific connective tissue diseases ctd. Interstitial pneumonia with autoimmune features show better survival. Rituximab therapy for interstitial pneumonia with autoimmune.
Interstitial pneumonia with autoimmune features ipaf ncbi. A through i, the idiopathic interstitial pneumonia iip meets interstitial pneumonia with autoimmune features ipaf criteria. Until now, only few data are available about the clinical and serological features of ipaf patients, their survival and the. Interstitial pneumonia with autoimmune features ipaf defines a distinct subset of patients with an idiopathic interstitial pneumonia iip. The european respiratory society american thoracic society proposed a term interstitial pneumonia with autoimmune features ipaf to further classify these individuals based on a combination of features from three.
Risk of progression of interstitial pneumonia with. Treatment of interstitial pneumonia with autoimmune features is an area of active study and there is no clear consensus. Ipaf interstitial pneumonia with autoimmune features interstitial pneumonia and features clinical, serological, andor morphological suggestive of a ctd that do not meet established classification criteria for a given autoimmune disease. The primary criticism expressed in the letter by collins and raghu is that the usual interstitial pneumonia. Patients with interstitial pneumonia with autoimmune features should be monitored by rheumatologists for development of systemic autoimmune rheumatic diseases. Critical assessment of this document and concept is essential to improving our understanding of this novel classification. Sep 23, 2019 interstitial pneumonia with autoimmune features an official european respiratory society american thoracic society research statement. Characterisation of patients with interstitial pneumonia with.
The interstitial pneumonia with autoimmune features criteria require the presence of an interstitial pneumonia on chest imaging or surgical lung biopsy, exclusion of an alternate cause, the. An easytoread bulleted format and state of the art imaging. Risk of progression of interstitial pneumonia with autoimmune. New definitions and diagnoses in interstitial pneumonia. A significant proportion of patients with interstitial lung disease ild manifest autoimmune features, but do not fulfill the diagnostic criteria for a definite connective tissue disease ctd. Download specialty imaging hrct of the lung 2e pdf part of the highly regarded specialty imaging series, this fully updated second edition by drs. Does interstitial pneumonia with autoimmune features represent a. Interstitial pneumonia with autoimmune features ipaf identifies a recently recognized autoimmune syndrome characterized by interstitial lung disease and autoantibodies positivity, but absence of a specific connective tissue disease diagnosis or alternative etiology. Idiopathic interstitial pneumonia iip with autoimmune features that does not fulfil connective tissue disease ctd criteria has been recently defined as interstitial.
Ipaf, interstitial pneumonia with autoimmune features. A through i, the idiopathic interstitial pneumonia iip meets interstitial pneumonia with autoimmune features. Patients with interstitial lung disease secondary to. Interstitial pneumonia with autoimmune features ncbi. To help define and study this disease entity more uniformly, a 2015 research statement proposed consensusbased criteria and coined the term interstitial pneumonia with autoimmune features ipaf. To overcome this issue, the referral centers for ild organized multidisciplinary teams mdts, including physicians and experts in. Interstitial pneumonia lung and airway disorders merck.
Interstitial pneumonia with autoimmune features ipaf prior studies have suggested differences in survival and clinical course for interstitial lung disease ild with specifically elicited clinical and serologic features of autoimmune disease. Patients who met the criteria for interstitial pneumonia with autoimmune features were excluded. Clinical profile and survival rate of interstitial lung disease in a tertiary care center of eastern nepal ghimire rh, ghimire a, bimali a, bista b department of pulmonary, critical care and sleep medicine. Interstitial pneumonia with autoimmune features american. Sep 18, 2017 interstitial pneumonia with autoimmune features ipaf identifies a recently recognized autoimmune syndrome characterized by interstitial lung disease and autoantibodies positivity, but absence of a specific connective tissue disease diagnosis or alternative etiology. Overall a ipaf cohort survival was significantly worse than the ctdild cohort p interstitial pneumonia with autoimmune features ipaf is a research classification proposed by the european respiratory societyamerican thoracic society task force on undifferentiated forms of connective tissue diseaseassociated interstitial lung disease as an initial step to uniformly define, identify, and study patients with interstitial lung. I then borrow from studies of connective tissue disease associated lung disease to make treatment selections. To our knowledge, there are no studies examining the efficacy of rituximab. Interstitial pneumonitis with autoimmune features ipaf.
Idiopathic interstitial pneumonia iip with autoimmune features that does not fulfil connective tissue disease ctd criteria has been recently defined as interstitial pneumonia with autoimmune features ipaf. Overall a ipaf cohort survival was significantly worse than the ctdild cohort p interstitial pneumonia with autoimmune features ipaf, which includes findings from the. Interstitial lung disease ild is a major cause of morbidity and mortality in patients with connective tissue diseases ctd. Interstitial pneumonia with autoimmune features ipaf was defined in 2015 by the working group of the european respiratory society ers and the american thoracic society ats as interstitial pneumonia with some clinical andor serological features suggesting presence of an underlying autoimmune. Does interstitial pneumonia with autoimmune features.
Researchers have proposed differing criteria and terms to describe these patients, and lack of consensus over nomenclature and classification limits the ability to conduct prospective. Its prevalence and prognosis remains controversial and seems to include a very heterogeneous population. A recent european respiratory societyamerican thoracic society statement proposed research criteria for interstitial pneumonia with autoimmune features ipaf, which includes findings from the. Mccoy ss, mukadam z, meyer kc, kanne jp, meyer ca, martin md, sampene e, aesif sw, rice ln, bartels cm. Gonzalez gay2, yannick allanore3 and marco matuccicerinic4,5. Patients with interstitial pneumonia with autoimmune features should be monitored by rheumatologists for development of systemic autoimmune. Crofford2 interstitial lung disease ild remains a cause of. This new classification system incorporates not only clinical and serological manifestations of ctd, but also morphological features suggestive of a ctd encountered on high. Although ild is well recognised in patients with established ctd, it is rarely the sole presenting feature of ctd. Pdf interstitial pneumonia with autoimmune features show better. Interstitial pneumonia with autoimmune features and. Patients with interstitial lung disease ild who show features related to autoimmunity without meeting criteria for a defined. A recent european respiratory societyamerican thoracic society research statement proposed criteria for patients with interstitial pneumonia with autoimmune features ipaf. It tends to affect healthy men and women who are usually older than 40.
Characterisation of patients with interstitial pneumonia. Interstitial pneumonia with autoimmune features ipaf is a research classification proposed by the european respiratory societyamerican thoracic society task force on undifferentiated forms of connective tissue diseaseassociated interstitial lung disease as an initial step to. See also overview of idiopathic interstitial pneumonias. Mycophenolate therapy in interstitial pneumonia with autoimmune. Background recently the term interstitial pneumonia with autoimmune features ipaf has been proposed to identify patients with interstitial lung disease and autoimmune characteristics, not fulfilling the criteria for specific connective tissue diseases ctd. A usual interstitial pneumonia uip pattern on chest ct scans is highly suggestive of uip pathologic findings. The task force published a research statement 11 outlining the interstitial pneumonia with autoimmune features ipaf concept and nomenclature along with a set of classification criteria table 1 to serve as a platform for future research of this iip subset. The task force introduced a novel entity termed interstitial pneumonia with autoimmune features ipaf. Pdf interstitial pneumonia with autoimmune features. Sep 18, 2017 interstitial pneumonia with autoimmune features ipaf is a rare autoimmune form of interstitial lung disease that can present acutely with ards and multiple organ failure, requiring icu admission and advanced life support measures included ecmo, if needed. Interstitial pneumonia with autoimmune features ipaf is a research classification proposed by the european respiratory societyamerican. Recently, the european respiratory societyamerican thoracic society proposed the interstitial pneumonia with autoimmune features ipaf criteria which represents the effort of pulmonologists to classify patients that may remain clinically undefined 1. Interstitial lung disease etiology known etiology unknown aka idiopathic unclassifiable autoimmune disease ra, ssc, sjogrens, iim environmental ild hypersensitivity pneumonitis occupational ild asbestosissilicosis druginduced ild amiomtxchemo smokingrelated desquamative interstitial pneumonia respiratory bronchiolitisild.
I, usual interstitial pneumonia with op white arrows. Overall a ipaf cohort survival was significantly worse than the ctdild cohort p interstitial pneumonia with autoimmune features ipaf is a rare autoimmune form of interstitial lung disease that can present acutely with ards and multiple organ failure, requiring icu admission and advanced life support measures included ecmo, if needed. Ct patterns were previously determined and included in the research registry as part of an ongoing study on the natural history of ild. The clear need to unify the nomenclature and criteria used to define this group of patients and to promote research prompted a working group of the american thoracic society ats and the european respiratory society ers to propose the term interstitial pneumonia with autoimmune features ipaf. Gonzalez gay2, yannick allanore3 and marco matuccicerinic4,5 affiliations. Patients with interstitial lung disease ild may have features of connective tissue disease ctd, but lack findings diagnostic of a specific ctd. A significant proportion of patients with interstitial lung disease ild manifest autoimmune features, but do not fulfill the diagnostic criteria for a. Under current guidelines, a uip pattern on ct images is specific for ipf after a thorough clinical and serologic workup has excluded other causes of interstitial lung disease ild. Interstitial pneumonia with autoimmune features ipaf is a recently proposed terminology for interstitial lung disease ild with evidence of autoimmunity that does not meet the criteria for a defined connective tissue disease ctd. An official european respiratory societyamerican thoracic. Interstitial pneumonia with autoimmune features ipaf is a term to describe individuals with both interstitial lung disease ild and combinations of other clinical, serologic, andor pulmonary morphologic features, which presumably originate from an underlying systemic autoimmune condition, but do not meet current rheumatologic criteria for a defined connective tissue disease ctd.
Kaplanmeier survival curves of interstitial pneumonia with autoimmune features ipaf, idiopathic pulmonary fibrosis ipf and connective tissue disease ctdinterstitial lung disease ild cohorts. Full text mycophenolate therapy in interstitial pneumonia. Kaplanmeier survival curves of interstitial pneumonia with autoimmune features ipaf, idiopathic pulmonary fibrosis ipf and connective tissue disease ctd interstitial lung disease ild cohorts. Acute interstitial pneumonia is an idiopathic interstitial pneumonia that develops suddenly and is severe. Multiple foci of op were present in tissue specimen. Interstitial lung disease ild may occur in the presence of autoimmune elements without meeting criteria for a distinctive connective tissue disease. Ipaf interstitial pneumonia with autoimmune features. Cohort diagram for a study aimed at characterizing outcomes in patients with interstitial pneumonia with autoimmune features ipaf. Interstitial pneumonia with autoimmune features clinical. A recent european respiratory societyamerican thoracic society statement proposed research criteria for interstitial pneumonia with autoimmune features ipaf, which includes. Interstitial pneumonia with autoimmune features confers an increased risk of developing a systemic autoimmune rheumatic disease. In 2015, the european respiratory society ers and american thoracic society ats task force on undifferentiated forms of connective tissue diseaseassociated interstitial lung disease proposed. Frontiers interstitial pneumonia with autoimmune features.
Interstitial lung disease ild is a group of many lung conditions. Interstitial pneumonia with autoimmune features ipaf is a research classification proposed by the european respiratory societyamerican thoracic society task force on undifferentiated forms of connective tissue diseaseassociated interstitial lung disease as an initial step to uniformly define, identify, and study patients with interstitial lung disease ild who have features of. Patients with interstitial lung disease ild who show features related to autoimmunity without meeting criteria for a defined connective tissue. Characterizing features of interstitial pneumonias. Interstitial pneumonia with autoimmune features an emerging challenge at the intersection of rheumatology and pulmonology erin m. Interstitial pneumonia with autoimmune features annals. A clinicopathologic, ultrastructural, and cell kinetic study. Abstract patients with interstitial lung disease ild may have features of connective tissue disease ctd, but lack findings diagnostic of a specific ctd.
Interstitial pneumonia with autoimmune features ipaf is a research classification proposed by the european respiratory societyamerican thoracic society task force on undifferentiated forms of connective tissue diseaseassociated interstitial lung disease as an initial step to uniformly define, identify, and study patients with interstitial. Interstitial lung diseases ilds often occur as a complication of connective tissue disease ctd. Interstitial pneumonia with autoimmune features ipafnsip. Acute interstitial pneumonia causes the same type of symptoms as the acute respiratory distress syndrome.
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