scholarly journals Interstitial Pneumonia With Autoimmune Features: Value of Histopathology

2017 ◽  
Vol 141 (7) ◽  
pp. 960-969 ◽  
Author(s):  
Ayodeji Adegunsoye ◽  
Justin M. Oldham ◽  
Eleanor Valenzi ◽  
Cathryn Lee ◽  
Leah J. Witt ◽  
...  

Context.— Patients with idiopathic interstitial pneumonia may display evidence of autoimmunity without meeting criteria for a defined connective tissue disease. A recent European Respiratory Society/American Thoracic Society statement proposed research criteria for interstitial pneumonia with autoimmune features (IPAF), which includes findings from the clinical, serologic, and morphologic domains. Objectives.— To investigate the importance of histopathologic criteria within the morphologic domain and to report our methodology for identifying these features. Design.— Patients with idiopathic interstitial pneumonia at the University of Chicago who underwent surgical lung biopsy or lung transplantation were assessed for IPAF histopathologic features, using the initial pathology interpretation in the electronic records. A focused rereview of available slides by a pulmonary pathologist was then performed for patients who failed to meet IPAF criteria on initial pathology assessment. Results.— Of 422 patients with idiopathic interstitial pneumonia, 176 (41.7%) underwent surgical lung biopsy or lung transplant. Forty-six of those 176 patients (26.1%) met IPAF criteria by initial pathology interpretation and a positive clinical or serologic feature. Of the remaining 130 patients, 73 (56.2%) met either the clinical or serologic domains without meeting the morphologic domain, whereas 36 (27.7%) had slides available for pathology rereview. This rereview demonstrated nonspecific interstitial pneumonia in 8 of 36 patients (22.2%) and lymphoplasmacytic infiltrates in 6 of 36 patients (16.7%), resulting in an additional 7 of 36 patients (19.4%) with idiopathic interstitial pneumonia that met the IPAF criteria. In IPAF, pulmonary vasculopathy was the most prevalent finding (45 of 84; 53.6%) and predicted increased mortality (hazard ratio, 2.5; P = .04). Conclusions.— Using a methodological approach to identifying IPAF pathology, we demonstrate a significant increase in the number of patients meeting IPAF criteria because of focused pathologic review and highlight the prognostic value of the IPAF pathologic findings.

2004 ◽  
Vol 11 (6) ◽  
pp. 437-440 ◽  
Author(s):  
D Jeffrey Moore ◽  
Colm P McParland ◽  
Martin J Bullock ◽  
Yannick Cartier ◽  
Paul Hernandez

The present case report describes a 44-year-old woman who presented with dyspnea due to diffuse interstitial lung disease. High-resolution computed tomography showed features of usual interstitial pneumonia, but the lung biopsy obtained by video-assisted thoracoscopy was consistent with a histological pattern of nonspecific interstitial pneumonia. Following the procedure, the patient developed progressive respiratory distress and died on postoperative day 13 with a clinical picture of acute interstitial pneumonia. The autopsy showed evidence of diffuse alveolar damage superimposed on the background pattern of nonspecific interstitial pneumonia. The present case report supports the notion that patients with a variety of subtypes of idiopathic interstitial pneumonias may be at risk of exacerbation of their underlying disease following thoracic procedures, including video-assisted thoracoscopic lung biopsy.


2016 ◽  
Vol 43 (5) ◽  
pp. 887-892 ◽  
Author(s):  
Sandra Chartrand ◽  
Jeffrey J. Swigris ◽  
Lina Peykova ◽  
Jonathan Chung ◽  
Aryeh Fischer

Introduction.Interstitial lung disease (ILD) is 1 possible manifestation of the idiopathic inflammatory myopathies (IIM). Occasionally, patients presenting with ILD are mistakenly diagnosed with idiopathic interstitial pneumonia (IIP), but after multidisciplinary evaluation, their ILD is determined to be because of antisynthetase syndrome (SynS) or myositis spectrum of disease.Methods.We used retrospective analytic methods to identify patients with ILD evaluated at the National Jewish Health between February 2008 and August 2014 and believed initially to have IIP but ultimately diagnosed with SynS or myositis spectrum of disease.Results.The cohort included 33 patients; most were white women with a mean age at presentation of 55 years. Their pulmonary physiologic impairment was moderate. In 31 cases, the ILD pattern by thoracic high-resolution computed tomography scan was nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), or a combination of the 2. Surgical lung biopsy was performed in 21 patients; NSIP was the most common pattern. Less than one-third of the cohort had positive antinuclear antibodies. Two-thirds had positive SSA. All patients had either myositis-specific or myositis-associated autoantibody. Most had subtle extrathoracic symptoms or signs of SynS; 12 had an elevated serum creatine phosphokinase, but none had clinical evidence of myositis. None met the Peter and Bohan classification criteria for polymyositis/dermatomyositis.Conclusion.Among patients who present with presumed IIP, a multidisciplinary evaluation that includes the integration of clinical evaluations by rheumatologists and pulmonologists, morphologic (both histopathologic and radiographic) data, and serologic features is helpful in the detection of occult SynS or the myositis spectrum of disease.


2006 ◽  
Vol 100 (10) ◽  
pp. 1753-1759 ◽  
Author(s):  
Yasuhiro Kondoh ◽  
Hiroyuki Taniguchi ◽  
Masanori Kitaichi ◽  
Toyoharu Yokoi ◽  
Takeshi Johkoh ◽  
...  

2016 ◽  
Vol 47 (6) ◽  
pp. 1767-1775 ◽  
Author(s):  
Justin M. Oldham ◽  
Ayodeji Adegunsoye ◽  
Eleanor Valenzi ◽  
Cathryn Lee ◽  
Leah Witt ◽  
...  

Patients with interstitial lung disease (ILD) may have features of connective tissue disease (CTD), but lack findings diagnostic of a specific CTD. A recent European Respiratory Society/American Thoracic Society research statement proposed criteria for patients with interstitial pneumonia with autoimmune features (IPAF).We applied IPAF criteria to patients with idiopathic interstitial pneumonia and undifferentiated CTD-ILD (UCTD). We then characterised the clinical, serological and morphological features of the IPAF cohort, compared outcomes to other ILD cohorts and validated individual IPAF domains using survival as an endpoint.Of 422 patients, 144 met IPAF criteria. Mean age was 63.2 years with a slight female predominance. IPAF cohort survival was marginally better than patients with idiopathic pulmonary fibrosis, but worse than CTD-ILD. A non-usual interstitial pneumonia pattern was associated with improved survival, as was presence of the clinical domain. A modified IPAF cohort of those meeting the clinical domain and a radiographic or histological feature within the morphological domain displayed survival similar to those with CTD-ILD.IPAF is common among patients with idiopathic interstitial pneumonia and UCTD. Specific IPAF features can identify subgroups with differential survival. Further research is needed to replicate these findings and determine whether patients meeting IPAF criteria benefit from immunosuppressive therapy.


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