Expert opinion and patients’ in-depth interviews on the impact of pulmonary complications in systemic sclerosis

Caminati, Antonella; Vigone, Barbara; Cozzaglio, Sergio; Nigris, Paola De; Galetti, Ilaria; Nunzio, Sara di; Verzeletti, Viviana; Cighetti, Jennifer; Garbagnati, Carla; Paleari, Laura; Tabaglio, Erminio; Pirri, Salvatore

doi:10.1080/03007995.2021.1992370

Cited by 5 publications

(2 citation statements)

References 19 publications

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“…Poiché l'incontro è avvenuto in modalità virtuale a causa della situazione pandemica, la metodologia è stata adattata (Figura 1). 33,34 Nominal Group Technique (NGT) (iv) ranking: ogni partecipante assegna in modo autonomo a ogni statement un punteggio tra 1 e 5 (1=non rilevante, 5=estremamente rilevante). I punteggi ottenuti per ciascuno statement vengono sommati, creando una graduatoria di idee giudicate maggiormente rilevanti sulla base di un processo di clustering.…”

Section: Metodiunclassified

La corretta gestione del paziente con amiloidosi ereditaria da transtiretina a fenotipo misto: le raccomandazioni di un gruppo di esperti Italiani

Canepa¹,

Cappelli²,

Limongelli³

et al. 2022

DIC

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Hereditary transthyretin amyloidosis (ATTRv) is a progressive systemic disease characterized by extracellular deposition of amyloid fibrils in different organs and tissues, leading to a sensory and motor polyneuropathy, often with the involvement of the autonomic nervous system, and infiltrative cardiomyopathy, with a fatal outcome within 4-15 years from the onset, if not treated. A cardiological phenotype, a neurological phenotype and a mixed phenotype, characterized by a worse outcome, are distinguished. In recent years, drugs acting on the amyloidogenesis process have increased. Consequently, the correct and timely diagnosis of ATTRv plays a central role. At the same time, it is essential to spread the knowledge of this disease and the related phenotype and elaborate recommendations for the correct patients' management, considering the limited literature evidence. These issues were addressed by a group of clinicians with proven experience in the management of ATTRv. The experts carried out a consensus activity through the Nominal Group Technique (NGT). This activity highlighted the importance of the differential diagnosis, placing genetic testing at the center of this process, and the relevance of a multidisciplinary approach to diagnosis and treatment, especially for the correct identification and management of patients with mixed phenotype. It was agreed that the main factors contributing to the definition of prognosis are genotype, neurological stage at diagnosis, cardiac involvement and rate of progression. During follow-up, each patient should undergo a complete evaluation of organ involvement through a multidisciplinary approach, especially useful for promptly verifying the onset of a mixed phenotype.

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Section: Metodiunclassified

La corretta gestione del paziente con amiloidosi ereditaria da transtiretina a fenotipo misto: le raccomandazioni di un gruppo di esperti Italiani

Canepa¹,

Cappelli²,

Limongelli³

et al. 2022

DIC

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“…Only a few studies have examined how patients and healthcare professionals (HCPs) experience and perceive diagnostic trajectories and the barriers related to the pre-diagnostic period in patients with ARD-ILD. Previous research has primarily focused on SSc-ILD [ 20 , 21 , 22 ].…”

Section: Introductionmentioning

confidence: 99%

The diagnostic trajectories of Danish patients with autoimmune rheumatologic disease associated interstitial lung disease: an interview-based study

Johansen

Bendstrup

Davidsen

et al. 2023

European Clinical Respiratory Journal

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Background Autoimmune rheumatologic disease associated interstitial lung diseases (ARD-ILD) are rare conditions and the association between ARDs and respiratory symptoms often goes unrecognised by ARD patients and general practitioners (GPs). The diagnostic trajectory from the first respiratory symptoms to an ARD-ILD diagnosis is often delayed and may increase the burden of symptoms and allow further disease progression.The aim of this study was to 1) characterise the diagnostic trajectories of ARD-ILD patients and to 2) identify barriers for obtaining a timely ILD diagnosis based on the experiences and perceptions of both patients and healthcare professionals. Method Semi-structured qualitative interviews were conducted with Danish ARD-ILD patients, rheumatologists, pulmonologists and ILD nurses. Results Sixteen patients, six rheumatologists, three ILD nurses and three pulmonologists participated. Five characteristics of diagnostic trajectories were identified in the patient interviews: 1) early referral to lung specialists; 2) early delay; 3) delay or shortcut depending on specific circumstances; 4) parallel diagnostic trajectories connected late in the process; 5) early identification of lung involvement without proper interpretation. With the exception of early referral to lung specialists, all of the diagnostic trajectory characteristics identified led to delayed diagnosis. Delayed diagnostic trajectories resulted in patients experiencing increased uncertainty. Inconsistent disease terminology, insufficient knowledge and lack of awareness of ARD-ILD among central healthcare professionals and delayed referral to ILD specialists were main contributors to the diagnostic delay identified by the informants. Conclusion Five characteristics of the diagnostic trajectories were identified, four of which led to diagnostic delay of ARD-ILD. Improved diagnostic trajectories can shorten the diagnostic trajectory and increase early access to appropriate specialist medical care. Improved awareness and expertise in ARD-ILD across different medical specialties, especially among GPs, may contribute to more efficient and timely diagnostic trajectories and improved patient experiences.

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Patient and caregiver shared experiences of pulmonary fibrosis (PF): A systematic literature review

Wells,

Harding,

Dixon

et al. 2024

Respiratory Medicine

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Expert opinion and patients’ in-depth interviews on the impact of pulmonary complications in systemic sclerosis

Cited by 5 publications

References 19 publications

La corretta gestione del paziente con amiloidosi ereditaria da transtiretina a fenotipo misto: le raccomandazioni di un gruppo di esperti Italiani

La corretta gestione del paziente con amiloidosi ereditaria da transtiretina a fenotipo misto: le raccomandazioni di un gruppo di esperti Italiani

The diagnostic trajectories of Danish patients with autoimmune rheumatologic disease associated interstitial lung disease: an interview-based study

Patient and caregiver shared experiences of pulmonary fibrosis (PF): A systematic literature review

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