The DIAMORFOSIS (DIAgnosis and Management Of lung canceR and FibrOSIS) survey: international survey and call for consensus

Abstract

BackgroundCurrently there is major lack of agreement on the diagnostic and therapeutic management of patients with Idiopathic Pulmonary Fibrosis (IPF) and lung cancer (LC). Our aim was to identify variations in diagnostic and management strategies across different institutions and provide rationale for a consensus statement on this issue.MethodsThis was a joint-survey by ERS Assemblies 8, 11 and 12. The survey consisted of 25 questions.ResultsFour hundred ninety four (n=494) physicians from 68 different countries and 5 continents responded to the survey. 94% of participants were pulmonologists and 1.8% thoracic surgeons and 1.9% oncologists. 97.7% involved MDT approaches on diagnosis and management. Regular low-dose HRCT scan was used by 49.5% of the respondents to screen for LC in IPF. PET scan and EBUS bronchoscopy is performed by 60% and 88%, to diagnose nodular lesions with mediastinal lymphadenopathy in patients with advanced and mild IPF, respectively. 83% of respondents continue anti-fibrotics following LC diagnosis; safety precautions during surgical interventions including low-tidal volume are applied by 67%. Stereotactic radiotherapy is used to treat patients with advanced IPF (DLCO<35%) and otherwise operable NSCLC by 54% of respondents and doublet platinum regimens and immunotherapy for metastatic disease by 25% and 31.9%, respectively. Almost all participants (93%) replied that a consensus statement for the management of these patients is highly warranted.ConclusionThe diagnosis and management of IPF-LC is heterogeneous with most respondents calling for a consensus statement.

Impact of idiopathic pulmonary fibrosis on clinical outcomes of lung cancer patients

“…Tzouvelekis et al, in 102 patients with IPF and lung cancer from a multicenter in Greece, reported that SqCC (34.3%) was the most common histologic subtype 35 . However, in a recent international survey performed by the ERS, participating physicians responded that adenocarcinoma (58.6%) was the most common histological type of lung cancer in patients with IPF, followed by SqCC (26.6%) 36 . In addition, the high proportion of male patients and lower lobe predominance are consistent with the results of previous studies 14 , 16 , 37 .…”

“…In our study, there were no differences in survival between the IPF and no-IPF groups among patients with SCLC or advanced (stage IV) NSCLC. A recent international survey reported that palliative care (69%) was the most frequent management in both advanced IPF and lung cancer (TNM stage IV) cases 36 . However, Koyama et al, in 122 patients with SCLC, reported that the IPF group (n = 20, median survival 244 days) had shorter survival than the IIP (idiopathic interstitial pneumonia) other than IPF group (n = 27, median survival 386 days) or non-IIP group (n = 73, median survival 592 days, P = 0.001) 42 .…”

Die idiopathische pulmonale Fibrose jenseits der Lunge: Krankheitsmechanismen verstehen, um Diagnose und Therapie zu verbessern

“…Platinhaltige Zweifach-Chemotherapie-Schemata und Immuntherapie bei metastasierter Erkrankung wurden von 25% bzw. 32% der Befragten angegeben [162].…”

Diagnostic and Therapeutic Challenges for Patients with Idiopathic Pulmonary Fibrosis and Lung Cancer

“…aspiration/biopsy (EBUS-TBNA/B) 19 . On the contrary, agreement rates were considerably decreased in cases of severe IPF 19 .…”

“…Current American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Latin American Thoracic Society (ALAT) guidelines do not address this crucial issue 1,7 . A recently published international survey, denominated the DIAMORFOSIS (DIAgnosis and Management Of lung canceR and FibrOSIS) survey, identified major heterogeneity in diagnostic and management strategies across different institutions and highlighted the amenable need for a consensus statement 19 . This review article aims to address the main diagnostic and therapeutic challenges for patients with IPF and lung cancer through a Q (questions) and A (answers) approach.…”