A scoping review of the unmet needs of patients diagnosed with idiopathic pulmonary fibrosis (IPF).

AIMS: Patients diagnosed with idiopathic pulmonary fibrosis (IPF) have a high symptom burden and numerous needs that remain largely unaddressed despite advances in available treatment options. There is a need to comprehensively identify patients' needs and create opportunities to address them. This scoping review aimed to synthesise the available evidence and identify gaps in the literature regarding the unmet needs of patients diagnosed with IPF. METHODS: The protocol for the review was registered with Open Science Framework (DOI 10.17605/OSF.IO/SY4KM). A systematic search was performed in March 2022, in CINAHL, MEDLINE, Embase, PsychInfo, Web of Science Core Collection and ASSIA Applied Social Science Index. A comprehensive review of grey literature was also completed. Inclusion criteria included patients diagnosed with IPF and date range 2011-2022. A range of review types were included. Data was extracted using a data extraction form. Data was analysed using descriptive and thematic analysis. A total of 884 citations were reviewed. Ethical approval was not required. RESULTS: 52 citations were selected for final inclusion. Five themes were identified: 1.) psychological impact of an IPF diagnosis. 2.) adequate information and education: at the right time and in the right way. 3.) high symptom burden support needs. 4.) referral to palliative care and advance care planning (ACP). 5.) health service provision-a systems approach. CONCLUSION: This review highlights the myriad of needs patients with IPF have and highlights the urgent need for a systems approach to care, underpinned by an appropriately resourced multi-disciplinary team. The range of needs experienced by patients with IPF are broad and varied and require a holistic approach to care including targeted research, coupled with the continuing development of patient-focused services and establishment of clinical care programmes.

An update on interstitial lung disease.

Interstitial lung diseases are a complex group of conditions that cause inflammation and scarring of the lung interstitium. This article discusses the diagnosis and management of common interstitial lung diseases including idiopathic pulmonary fibrosis, hypersensitivity pneumonitis, connective tissue disease associated-interstitial lung disease, sarcoidosis and drug-induced interstitial lung disease. A multidisciplinary approach to diagnosis of interstitial lung disease is the gold standard; key history and examination features, blood panel, pulmonary function tests, high resolution computed tomography imaging, and when required bronchoalveolar lavage and lung biopsy results are discussed to reach a multidisciplinary consensus diagnosis. Advances, including the development of the disease-modifying anti-fibrotic medications nintedanib and pirfenidone, continue to shape the future management of interstitial lung disease. A holistic approach to the care of patients with interstitial lung disease is paramount, as they often have a high symptom burden and considerable palliative care needs.

Care programs and their components for patients with idiopathic pulmonary fibrosis: a systematic review.

BACKGROUND: The multidimensional and complex care needs of patients with idiopathic pulmonary fibrosis (IPF) call for appropriate care models. This systematic review aimed to identify care models or components thereof that have been developed for patients with IPF in the outpatient clinical care, to describe their characteristics from the perspective of chronic integrated care and to describe their outcomes. METHODS: A systematic review was conducted using state-of-the-art methodology with searches in PubMed/Medline, Embase, CINAHL and Web Of Science. Researchers independently selected studies and collected data, which were described according to the Chronic Care Model (CCM). RESULTS: Eighteen articles were included describing 13 new care models or components. The most commonly described CCM elements were 'delivery system design' (77%) and 'self-management support' (69%), with emphasis on team-based and multidisciplinary care provision and education. The most frequently described outcome was health-related quality of life. CONCLUSIONS: Given the high need for integrated care and the scarcity and heterogeneity of data, developing, evaluating and implementing new models of care for patients with IPF and the comprehensive reporting of these endeavours should be a priority for research and clinical care.

Multiple Traditional Chinese Medicine interventions for idiopathic pulmonary fibrosis: A protocol for systematic review and meta-analysis of overview.

BACKGROUND: The therapeutic strategies of idiopathic pulmonary fibrosis (IPF) tend to be comprehensive. Improving the major symptoms and quality of life (QoL) is as important as postponing the process of fibrosis. However, only pirfenidone and nintedanib conditionally recommended by guidelines and no definite proof indicate that they can significantly ameliorate the main symptoms and QoL of IPF sufferers. At present, multiple types of Traditional Chinese Medicine (TCM) interventions alone or in combination with conventional western medicine managements are widespreadly applied in IPF treatment, which seemingly have a promising clinical effect, especially in ameliorating the main symptoms and improving QoL. Subsequently, the number of relevant studies in systematic reviews(SRs) and meta-analyses of randomized controlled trials(RCTs) increased significantly. Hence, we plan to implement an overview to collect, evaluate, and summarize the results of these SRs. METHODS: An all-round literature retrieval will be conducted in 9 electronic databases, including PubMed, EMBASE, CINAHL, Cochrane Library, Epistemonikos, CNKI, CBM, Wanfang, and VIP. We will focus on the systematic review and meta-analysis of RCTs for multiple TCM interventions alone or in combination with routine western medicine measures in IPF treatment. The main outcomes we follow with interest include the improvement of major symptoms (cough, dyspnea) and QoL. Secondary outcomes will consist of minor symptoms improvement, clinical total effective rate, lung function, blood gas analysis, 6-minute walk text, adverse events, acute exacerbation, all-cause mortality, and IPF-related mortality. Two reviewers will independently select the SRs satisfactory with the enrolling criteria, extract key characteristics, and datas on predefined form, evaluate methodological quality by AMSTAR-2, ROBIS and PRISMA tools, and the quality of evidences adopting GRADE method. In case of any divergence will be reached an agreement by discussion or adjudicated by a third senior reviewer. We will perform a narrative synthesis of the proofs from SRs included. RESULTS: The findings of this overvew will be presented at relevant conferences and submitted for peer-review publication. CONCLUSIONS: We expect to obtain comprehensive and reliable evidence of IPF treated by diversified TCM interventions from the potential standard SRs, which may provide suggestions for future RCTs and SRs. REGISTRATION NUMBER: INPLASY 202080110.

Long-Term Combined Training in Idiopathic Pulmonary Fibrosis: A Case Study.

A supervised combined training program was applied to a sedentary 56-year-old man with idiopathic pulmonary fibrosis (IPF) along three years, until lung transplantation. It included: (a) aerobic continuous (CT) and interval training (IT), (b) high load resistance training (RT) and (c) inspiratory muscle training (IMT). IT and IMT were applied for two years, while CT and RT could be maintained until transplantation using supplemental oxygen. Maximal inspiratory pressure (MIP) kept above 180 cm H2O and forced vital capacity (FVC) remained stable until lung transplantation. Peak oxygen uptake VO2 increased during 1.5 years before its decline, staying above the poor prognosis level two years. Finally, the patient maintained his walking capacity and independence for 2 years, before the decline due to the disease. After receiving a two-lung transplant, the patient remained intubated for 12 h, left the intensive care unit after 3.5 days and was discharged after 18 days (average values: 48 h, 7-10 days and 25-35 days, respectively). These results show that systematic and supervised combined training can be safety applied in an IPF patient to maintain functionality and quality of life. In addition, we show that RT can be maintained for as long as necessary without complications.

Health Care Costs at the End of Life for Patients with Idiopathic Pulmonary Fibrosis. Evaluation of a Pilot Multidisciplinary Collaborative Interstitial Lung Disease Clinic.

Rationale: Even though idiopathic pulmonary fibrosis (IPF) is a disease with high morbidity and mortality and no cure, palliative care is rarely implemented, leading to high symptom burden and unmet care needs. In 2012, we implemented a multidisciplinary collaborative (MDC) care model linking clinic and community multidisciplinary teams to provide an early integrated palliative approach, focusing on early symptom management and advance care planning.Objectives: To evaluate the differences in resource use and associated costs of end-of-life care between patients with IPF who received early integrated palliative care and patients with IPF who received conventional treatment.Methods: Using administrative health data, we identified all patients in the Province of Alberta, Canada, who presented to a hospital with an IPF diagnosis between January 1, 2012, and December 31, 2018, and died within this time frame. We compared three groups of patients: those who received MDC care (our clinic patients), specialist care (SC; respirologist), or non-specialist care (NSC; no contact with a respiratory clinic). The primary outcomes were healthcare resource use and costs in the year before death.Results: Of 2,768 patients across the three study groups, in the last year of life, MDC patients were more than three times as likely as SC patients to have received antifibrotic therapies (odds ratio [OR], 3.0; 95% confidence interval [CI], 1.8-5.2), almost twice as likely to have received pulmonary rehabilitation (OR, 1.9; 95% CI, 1.1-3.4), and 36% more likely to have received opiates (OR, 1.4; 95% CI, 0.8-2.3). The median total healthcare costs in the last 3 months of life were approximately C$7,700 lower for MDC patients than for those receiving SC, driven primarily by fewer hospitalizations and emergency department visits. MDC patients were also less likely to die in the hospital (44.9% MDC vs. 64.9% SC vs. 66.8% NSC; P < 0.001) and had the highest rates of no hospitalization in the last year of life.Conclusions: An integrated palliative approach in IPF is associated with improvements in the quality of end-of-life care and reduction in costs. Transformation of care models is required to deliver palliative care for patients with IPF. MDC teams within such models can address the high burden of unmet needs for symptom management, advance care planning, and community support in this complex population.

Efficacy of TCM therapy of tonifying lung-kidney's Qi-deficiency in a case of idiopathic pulmonary fibrosis: A case report.

RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a special form of spontaneous, chronic, progressive interstitial fibrotic pneumonia of unknown cause, and treatments for IPF have shown a poor prognosis. This study reports a new treatment, traditional Chinese medicine (TCM) therapy, for tonifying Qi-deficiency of lung-kidney in a 64-year-old patient with IPF. PATIENT CONCERNS: A 64-year-old man, who complained of cough and asthma, was diagnosed as IPF with mild impairment in lung function by thoracic high-resolution computed tomography and pulmonary function test. He received an 18-month N-acetylcysteine monotherapy but had no improvement in lung function. DIAGNOSES: IPF with mild impairment in lung function was diagnosed. INTERVENTIONS: The Chinese herbal medicine composition was decocted in 300 ml water for oral administration with 150 ml decoction twice daily in June 2017. OUTCOMES: The pulmonary function test showed that diffusing capacity for carbon monoxide had increased to 81% of predicted back to normal after 2-month TCM monotherapy. And diffusing capacity for carbon monoxide had increased to 89% of predicted, and forced expiratory volume in 1 s/forced vital capacity ratio increased to 92% at 14-month follow-up. No adverse events occurred during the 14 months of therapy and observation. LESSONS: The treatment by TCM therapy of tonifying lung-kidney's Qi-deficiency for IPF can improve the pulmonary function and reverse disease progression; it may be considered as a complementary treatment for IPF with mild-to-moderate impairment. However, the insights provided in this case report require further exploration and verification.

Multipotent Mesenchymal Stromal Cells for Pulmonary Fibrosis?

With the combination of ideologic beliefs and the will to survive, fraught patients and determined clinicians seek alternative therapies for treatment of terminal conditions, such as idiopathic pulmonary fibrosis. Unfortunately, the need for treatment has supported the growth of unapproved stem cell therapy over the years spanning across many countries. The reality, however, is that the science behind this therapy is lagging. While there have been promising results from phase I trials, there remain multiple reasons that "stem cells" are not ready for clinical application, starting from a gap in understanding at the bench research level, all the way to optimal clinical application in order to provide effective therapy.

[Umbilical moxibustion for patients with idiopathic pulmonary fibrosis complicated with gastroesophageal reflux of lung-spleen qi deficiency].

OBJECTIVE: To evaluate the clinical efficacy of umbilical moxibustion in the treatment of patients with idiopathic pulmonary interstitial fibrosis complicated with gastroesophageal reflux of lung-spleen qi deficiency. METHODS: A total of 116 patients with idiopathic pulmonary fibrosis complicated with gastroesophageal reflux of lung spleen-deficiency were randomized into an observation group (58 cases) and a control group (58 cases). In the control group, the routine treatment was applied; in the observation group, on the base of the treatment as the control group, the umbilical moxibustion was used. The treatment was given 2 times a week, 2 h each time for 12 weeks, and followed up for 12 weeks after treatment. The composite physiological indicators (CPI), TCM syndrome scores, the St. George respiratory questionnaire score (SGRQ) and 6-minute walk test (6MWT) were compared before and after treatment in the two groups. RESULTS: The improvement of the TCM syndrome scores (cough, post-activity shortness of breath, fatigue, heartburn, acid reflux), CPI score and 6MWT in the observation group was better than those in the control group (all P<0.05). There was no significant difference in the scores of SGRQ scale between the two groups after treatment (P>0.05). There was no significant difference in the scores of CPI scores and syndromes (cough and fatigue) between the two groups at 12 weeks after treatment (all P>0.05). The 6MWT, SGRQ scale scores and syndrome scores (post-activity shortness of breath, acid reflux, heartburn) were better than those in the control group in the follow up (all P<0.05). CONCLUSION: Umbilical moxibustion can improve the ventilation and diffusion function of lung in patients with idiopathic pulmonary fibrosis and gastroesophageal reflux of lung-spleen qi deficiency, reduce clinical symptoms and improve patients' quality of life and activity endurance. And it has long-term effect.

Prolonged Survival in a Patient with Idiopathic Pulmonary Fibrosis Receiving Acupuncture and DHEA-Promoting Herbs with Conventional Management: A Case Report.

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a lethal, chronic, degenerative disease most commonly affecting the elderly population. The pathologic finding of this restrictive lung condition is excessive deposition of fibrotic tissue in the alveolar interstitium. Conventional therapies minimally alter the course of disease, and mean survival time from diagnosis is only 2 to 4 years. CASE PRESENTATION: A 65-year-old man, diagnosed with IPF in 2007, was being monitored by a conventional pulmonologist while being treated with weekly acupuncture targeting a Chinese medicine diagnosis of spleen dampness and lung qi weakness and with botanical medicine targeting the stimulation of adrenal dehydroepiandrosterone secretion. He survived for 10 years after diagnosis. DISCUSSION: Studies exist linking dehydroepiandrosterone with IPF disease activity as well as acupuncture with antifibrotic activity. Larger clinical studies are needed to substantiate the link between acupuncture and/or dehydroepiandrosterone-stimulating herbs for the treatment of IPF.

Effectiveness and Safety of Chinese Medicine for Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.

OBJECTIVE: To evaluate the effectiveness and safety of Chinese medicine (CM) for Idiopathic pulmonary fibrosis (IPF) patients. METHODS: To screened relevant articles, PubMed, Cochrane Library, Excerpta Medica Datase (EMBASE), China National Knowledge Infrastructure (CNKI), Chinese VIP Information (VIP), Wanfang Database and Chinese Biomedical Database (CBM) were searched in English or Chinese until December 2015 for randomized controlled trials, which compared CM treatment (CM group) with Western medicine or placebo (control group) on IPF. The outcome measures included acute exacerbation, pulmonary function, the St George's respiratory questionnaire (SGRQ) scores, 6-minute walk test (6MWT) distance, adverse events and mortality. RESULTS: This meta-analysis included 25 randomized controlled trials involving 1,471 patients. Compared with the control group, CM group was superiori in reducing the risk of exacerbation [relative risk (RR)=0.40, 95% CI 0.22 to 0.72, P<0.05], improving in forced expiratory volume in one second (FEV1) [standard mean difference (SMD)=0.62, 95% CI 0.40 to 0.84, P<0.01] and diffusion capacity for carbon monoxide (DLCO, SMD=0.40, 95% CI 0.22 to 0.58, P<0.01), but there was no significant difference in vital capacity (VC, SMD=0.10, 95% CI-0.12 to 0.31, P>0.05). This meta-analysis also revealed that CM therapy significantly decreased the SGRQ score (SMD=-0.60, 95% CI-1.14 to-0.05, P<0.05) and improved 6MWT distance (SMD=0.59, 95% CI 0.34 to 0.84, P<0.01), compared with the control group. Meanwhile, CM therapy was associated with a low incidence of adverse effects (RR=0.19, 95% CI 0.08 to 0.43, P<0.01). However, there was no significant difference in mortality (RR=0.24, 95% CI 0.05 to 1.10, P>0.05) between CM and control groups. CONCLUSIONS: The pooled outcomes suggest that CM treatment appears benefit in reducing the risk of exacerbation, improving lung function and decreasing the incidence of adverse effects and enhancing the quality of life. However, the outcomes were limited because of the low quality of the included studies. More rigorous clinic trials need to be carried out to provide sufficient and accurate evidence in the future.

Early integrated palliative approach for idiopathic pulmonary fibrosis: A narrative study of bereaved caregivers' experiences.

BACKGROUND: Idiopathic pulmonary fibrosis has an uncertain and rapid trajectory after diagnosis. Palliative care is rarely utilized, although both patients and caregivers experience a distressingly high symptom burden. Most patients die in hospital. AIM: The purpose of this study was to explore bereaved caregivers' experiences and perceptions of an early integrated palliative approach implemented at a Multidisciplinary Interstitial Lung Disease Clinic. DESIGN: A narrative approach was used, with thematic and content analysis of open-ended interviews. SETTING/PARTICIPANTS: The clinic is located in a large western Canadian city. Caregivers of deceased patients were recruited through purposive sampling. The eight participants were either spouses or adult children. RESULTS: Five major themes were identified: Having a Terminal Disease; Planning Goals and Wishes for Care; Living Life and Creating Memories; Feeling Strain and Responsibility; and Nearing the End. Caregivers had little understanding of prognosis prior to advance care planning conversations at the clinic. Advance care planning conversations enabled caregivers to know and support patients' goals and wishes. Caregivers described feeling informed, prepared, and supported when death was near. They expressed neither distress nor anxiety related to patients' symptoms or strain of relationships. CONCLUSION: Collaboration and close communication among caregivers, respirologists, and home care enabled effective symptom management and out of hospital deaths. Patients and caregivers had opportunities to enjoy events, create memories, determine preferences, and make plans. Further research on an early integrated palliative approach in Idiopathic Pulmonary Fibrosis is warranted related to quality of life, experience with death and dying, and caregiver bereavement.

Beyond Idiopathic Pulmonary Fibrosis Diagnosis: Multidisciplinary Care With an Early Integrated Palliative Approach Is Associated With a Decrease in Acute Care Utilization and Hospital Deaths.

CONTEXT: Idiopathic pulmonary fibrosis (IPF) is a progressive, incurable interstitial lung disease with heavy symptom burden and poor quality of life. The last year of life is characterized by increased acute care utilization and hospital deaths. Clinical guidelines recommend early integration of palliative care but are rarely implemented. In 2012, we reorganized our clinic into a multidisciplinary team comprising two pulmonologists (expertise in interstitial lung disease and palliative respiratory care, respectively), nurse, respiratory therapist, physiotherapist, and a dietitian. We adopted an early integrated palliative approach with a focus on early symptom management and advance care planning starting at the first clinic visit. We designed a Multidisciplinary collaborative (MDC) care model with emphasis on community-based care to manage patients in their homes and support caregivers. OBJECTIVES: Exploratory analysis of this model's association with acute care utilization in the last year of life and location of death was undertaken. METHODS: Data from deceased IPF patients before and after 2012 (non-MDC and MDC care model, respectively) were collected, and statistical analysis was performed. RESULTS: Patients in MDC care were 24.2 times less likely to have respiratory-related emergency room visits (95% CI: 3.12-187.44, P = 0.002), 2.32 times less likely to have respiratory-related hospitalizations (95% CI: 0.95-5.6, P = 0.064). The odds of achieving a home or hospice death in MDC care were 9.2 times compared to non-MDC care, who die mostly in the hospital (95% CI: 1.14-75, P = 0.037). CONCLUSIONS: MDC care model for IPF was associated with reduced health care use in the last year of life and more home deaths.

Acupuncture for idiopathic pulmonary fibrosis: Protocol for a systematic review.

BACKGROUND: Patients with idiopathic pulmonary fibrosis (IPF) often suffer from progressive worsening of dyspnea and lung function. Although 2 drugs are now available for IPF treatment, many gaps are still to be filled. Evidences suggest that there may be some beneficial effect of acupuncture on pulmonary diseases. This review aims to systematically determine the efficacy and safety of acupuncture for treating IPF. METHODS: The following 8 databases will be searched from their inception to March 20, 2017: PubMed, Embase, Cochrane Central Register of Controlled Trials, Web of Science, Chinese Biomedical Literature Database, China National Knowledge Infrastructure, Chongqing VIP, and Wanfang Data. Randomized controlled trials (RCTs) of acupuncture for IPF without restriction of publication status will be included. Two review authors will independently undertake study selection, extraction of data, and assessment of study quality. Meta-analysis will be conducted after screening of studies if possible. Dichotomous data will be analyzed using odds ratio (OR), and continuous data mean difference (MD). Statistical analysis will be conducted using Review Manager (RevMan) software. RESULTS: This systematic review will provide a detailed summary of the current evidences related to the efficacy and safety of acupuncture in improving breathlessness, exercise limitation, health status impairment of patients with IPF. CONCLUSION: This evidence may be useful to clinicians, patients, and health policy-makers with regard to the use of acupuncture in IPF treatment. ETHICS AND DISSEMINATION: This systematic review will provide up-to-date information on acupuncture for IPF. This review does not require ethical approval and will be disseminated electronically through a peer-reviewed publication or conference presentations. TRIAL REGISTRATION NUMBER: PROSPERO 2017:CRD42017059848.

European IPF Patient Charter: unmet needs and a call to action for healthcare policymakers.

Patient advocacy groups play an important role in supporting patients with chronic diseases and promoting better care. The aim of this patient-physician initiative was to gather perceptions from European idiopathic pulmonary fibrosis (IPF) patient advocacy groups regarding inequalities and unmet needs in IPF care, in order to develop a Patient Charter to advocate for better care.In total, 11 European patient advocacy groups were interviewed regarding the care of patients with IPF in their countries. Interview feedback was presented to a Working Group including patient advocacy group representatives and IPF specialists; key areas of agreement were developed into the European IPF Patient Charter.The interviews identified five key themes that fed into the final Charter: the need for improved diagnosis, treatment access, holistic care, disease awareness and palliative care. The final Charter was endorsed by patient advocacy groups and presented to 26 Members of the European Parliament in September 2014. It has received >8900 signatures to date.This patient-physician initiative highlights the inequalities and unmet needs in IPF care across Europe, and demonstrates how this insight can inform the development of a Patient Charter, designed as a call to action for healthcare policymakers to drive improvement in European IPF care.

Fibrosis pulmonar idiopática / Idiopathic pulmonary fibrosis

La FPI predomina en el sexo masculino, en edades avanzadas, con tos y/o disnea progresivas. Un 5% se presenta como una forma familiar. La tomografía axial computarizada de tórax, fundamental en el diagnóstico, en al menos un 50% hace innecesaria la biopsia. El diagnóstico es conjunto con clínicos, radiólogos y patólogos. La sobrevivencia media es de tres a cinco años desde el diagnóstico. La historia natural es un deterioro progresivo, pero hay formas rápidas y también pueden aparecer exacerbaciones que ensombrecen el pronóstico. Diversas comorbilidades se han descrito como la hipertensión pulmonar, la asociación con enfisema y el reflujo gastroesofágico. Sólo recientemente aparecen fármacos útiles, que son la Pirfenidona y el Nintedanib. El clásico esquema de prednisona, azatriopina y N-acetil cisteina, se ha demostrado ineficaz. Otros recursos que pueden utilizarse como complementos útiles en la enfermedad son el oxígeno, la rehabilitación, las terapias antirreflujo y el manejo sintomático de la tos.

PF appears mainly in aged males, with progressive cough and dyspnea. In 5% of the cases the disease presents as a familial form. CT scan is key in diagnosis of the disease. In no less than 50% biopsy is unnecessary but diagnosis must be made in conjunction with clinician, radiologist and pathologist. Median survival is 3 to 5 years from diagnosis. Natural history is a progressive deterioration but there are fast evolution cases and exacerbation of the disease that make worse the prognosis. Pulmonary hypertension, the association with emphysema and gastroesophageal reflux has been described as comorbidities of the disease. Last year has been published the positive results of therapeuticall trials with two new drugs, Pirfenidone and Nintedanib. The classical regime for IPF with Prednisone, Azathriopine and Acetylcysteine has been showed as useless. Oxygen, Pulmonary rehabilitation, gastroesophageal reflux and cough management are complementary treatment for the disease.

[Idiopathic pulmonary fibrosis: modern guideline-concordant diagnostics and innovative treatment]. / Idiopathische Lungenfibrose - moderne, leitliniengerechte Diagnostik und innovative Therapien.

Interstitial lung diseases are a heterogenic group of diseases who share in common manifestation of the disease process in the distal airways and alevolo-capilary membrane. Idiopathic pulmonary fibrosis (IPF) is of special interest as it is the most prevalent entity within the idiopathic interstitial pneumonias and it is associated with the worst prognosis. Diagnosis of IPF necessitates confirmation of a "usual interstitial pneumonia" (UIP) pattern on high-resolution computed tomography or on histology from a surgical lung biopsy. The recently published evidence-based IPF-guidelines for the first time define radiologic criteria for a UIP pattern and thus allow to make an IPF diagnosis in the absence of a surgical lung biopsy. Moreover, an IPF diagnosis can be made only after exclusion of any other cause for an interstitial lung disease.In the new IPF-guideline no medical therapy has received a positive recommendation. Based on the available evidence only pirfenidone has been shown to attenuate disease progression in mild to moderate IPF populations, with low-moderate quality of evidence. Pirfenidone has been approved for mild-moderate IPF in the European Union in February 2011 and is approved in Japan since 2009. In IPF-patients who cannot receive pirfenidone high-dose N-acetylcysteine is an "off-label-use" option that also has been shown to slow disease progression, however, based on low quality of evidence only. Recent studies suggest that anti-inflammatory therapies (prednione, azathioprine) may be harmful in IPF patients and cannot be recommended. Besides medical therapies IPF patients should receive best supportive care, which includes long-term oxygen therapy in hypoxemic patients and pulmonary rehabilitation as well as lung transplantation for eligible patients.

Comprehensive care of the patient with idiopathic pulmonary fibrosis.

PURPOSE OF REVIEW: Recently, an expert committee endorsed by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and the Latin American Thoracic Society published an evidence-based guideline on the management of idiopathic pulmonary fibrosis (IPF). In the current document, we summarize and supplement this recent expert document and propose a comprehensive approach to the care and management of patients with IPF. RECENT FINDINGS: We propose three pillars of care for the patient with IPF titled 'disease-centered management', 'symptom-centered management', and 'education and self-management'. Disease-centered management involves both pharmacological and nonpharmacological approaches. Palliative care should be an integral and routine component of the care of patients with IPF. Education and self-management strengthens the provider-patient partnership by enabling patients to set realistic goals, remain in control of his or her care, and prepare for the future. SUMMARY: The comprehensive care of the patient with IPF involves balancing the three pillars of disease-centered management, symptom-centered management, and patient education and self-management upon a solid foundation of provider-patient partnership. Constant reassessment of the individual patient's goals of care, based on their values and preferences, is essential to the constant recalibration of these various interventions.