Some take-home messages from the 9th International Meeting on Pulmonary Rare Diseases and Orphan Drugs

Dissemination of updated knowledge on rare and ultra-rare respiratory diseases has always been a priority of the European Respiratory Review (ERR) [1, 2]. In this issue, we publish the first of a collection of four articles that discuss hot topics with direct practical clinical consequences. First, Mackintosh et al. [3] review the challenges and controversies (which are not few!) in “interstitial pneumonia with autoimmune features” (IPAF). Following the publication, in 2015, of the classification of IPAF proposed by the European Respiratory Society (ERS) and the American Thoracic Society (ATS) [4], a number of articles have been published on this entity which truly requires a multidisciplinary approach. Airway and vascular involvement are discussed in detail, as well as the problem of severe vasculopathy that can characterise some IPAF patients. The authors also discuss the controversial issue of the therapeutic approach for this “research entity” and state that “while identification of a histological NSIP pattern would support an immunosuppressive approach, histological confirmation of a UIP pattern would lead to a more cautious approach to immunosuppression, with frequent monitoring and earlier consideration of anti-fibrotic therapy, should there be progression” [3].

Short abstract

The 9th International Meeting on Pulmonary Rare Diseases and Orphan Drugs was dedicated to the management of rare pulmonary parenchymal and vascular diseases and rare variants of more common respiratory conditions https://bit.ly/3rq7b95

Dissemination of updated knowledge on rare and ultra-rare respiratory diseases has always been a priority of the European Respiratory Review (ERR) [1, 2]. In this issue, we publish the first of a collection of four articles that discuss hot topics with direct practical clinical consequences. First, Mackintosh et al. [3] review the challenges and controversies (which are not few!) in “interstitial pneumonia with autoimmune features” (IPAF). Following the publication, in 2015, of the classification of IPAF proposed by the European Respiratory Society (ERS) and the American Thoracic Society (ATS) [4], a number of articles have been published on this entity which truly requires a multidisciplinary approach. Airway and vascular involvement are discussed in detail, as well as the problem of severe vasculopathy that can characterise some IPAF patients. The authors also discuss the controversial issue of the therapeutic approach for this “research entity” and state that “while identification of a histological NSIP pattern would support an immunosuppressive approach, histological confirmation of a UIP pattern would lead to a more cautious approach to immunosuppression, with frequent monitoring and earlier consideration of anti-fibrotic therapy, should there be progression” [3].

Second, Bandyopadhyay et al. [5] address the challenge of the perioperative management of patients with precapillary pulmonary hypertension (PH) undergoing noncardiac non-obstetric surgery. When compared with elective surgery, emergency noncardiac procedures are associated with more perioperative complications and increased likelihood of worse outcome [6]. Haemodynamic ventricular interdependence and the possible causes of right ventricular failure and of its prevention are addressed in detail. The use of inotropes and of other drugs is a critical issue that is also reviewed carefully, together with the intraoperative management and monitoring, as well as ventilation and post-operative care.

Third, a forthcoming article by S. Tomassetti and co-workers reviews the challenging and poorly understood topic of interstitial lung abnormalities and its possible progression to idiopathic pulmonary fibrosis or to progressive fibrosing interstitial lung disease. Radiological definition and clinical implications are reviewed in detail.

The final article was written by a multidisciplinary group of experts invited by the World Association of Sarcoidosis and other Granulomatous Disorders (WASOG). In this WASOG statement on the diagnosis and management of sarcoidosis-associated PH, Savale et al. [7] present updated information on this severe complication of sarcoidosis. This statement is timely in the context of the recent publication of the ERS clinical practice guidelines on treatment of sarcoidosis [8–10].

Several of these articles derive from invited lectures and debates which took place during the digital ninth International Meeting on Pulmonary Rare Diseases and Orphan Drugs. This conference was endorsed by the European Reference Network on rare respiratory diseases (ERN-LUNG), a network of healthcare providers dedicated to the care and investigation of patients suffering from rare respiratory diseases (https://ec.europa.eu/health/ern/networks_en) [11]. This international conference, held every other year, is entirely devoted to the management of rare pulmonary parenchymal and vascular diseases and rare variants of more common respiratory conditions such as severe refractory asthma. We thank the ERR for once again publishing articles from this conference and hope that our readers will enjoy this selection, we are hopeful the 10th international meeting will be hosted face-to-face in 2023.