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Purpose of Review: Sarcoidosis is a multiorgan system disease exerting significant impact on biophysical, social, psychological and emotional well-being. Mortality and disability correlate to accessible, timely, expert care for sarcoidosis and its related complications. Across health conditions, positive healthcare interactions and interventions can rehabilitate unfavourable factors tied to concepts of ' frailty' . Here, we set out to introduce concepts related to frailty and their impact in the context of sarcoidosis., Recent Findings: Studies examining frailty across other multiorgan and single organ-based diseases that mirror organ involvement in sarcoidosis demonstrate findings that bear relevance in sarcoidosis. Namely, factors predisposing a person to frailty are a multifactorial phenomenon which are also reflected in the lived experience of sarcoidosis; and that early diagnosis, intervention and prevention may alter a course towards more favourable health outcomes., Summary: Factors predisposing to frailty in other health conditions may also signal a risk in sarcoidosis. In turn, proactive health preservation - regardless of age - may lead to improved biopsychosocial reserve and health-related quality of life. Fortifying holistic resilience in sarcoidosis is anticipated to reduce risk of the occurrence and prolongation of health-related complications, and facilitate swifter recovery from biophysical complications as well as from psychosocial and emotional stressors., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose of Review: Sarcoidosis is a chronic multisystemic inflammatory disease of unknown aetiology with a wide range of highly variable clinical manifestations and unpredictable disease course. Sarcoidosis patients may present with specific organ-related symptoms involving functional impairments, and less specific symptoms. The decision whether and when to treat a sarcoidosis patient with pharmacotherapy depends on two major factors: risk of organ failure and/or death and impairment of quality of life. This decision is complex and not standardized., Recent Findings: Glucocorticoids (GCs) are recommended as initial treatment, when needed. Subsequent GC-sparing alternatives frequently follow. Comorbidities or adverse drug reactions (ADRs) from drugs used in sarcoidosis treatment are sometimes very hard to differentiate from symptoms associated with the disease itself, which may cause diagnostic dilemmas. An ideal approach to minimalize ADRs would involve genetic screening prior to prescribing certain 'high-risk drugs' and therapeutic drug monitoring during treatment. Pharmacogenomic testing aims to guide appropriate selection of medicines, with the potential of reducing unnecessary polypharmacy while improving clinical outcomes., Summary: A multidisciplinary approach to the management of sarcoidosis may avoid unnecessary ADRs. It is important to consider the possibility of drug-induced damage in sarcoidosis, especially if the clinical situation deteriorates after the introduction of a particular drug., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose of Review: Drug use in elderly people is high compared to younger people. Simultaneously, elderly are at greater risk when exposed to environmental substances. It is puzzling therefore, that ageing, as a variable in pharmacological and toxicological processes is not investigated in more depth. Moreover, recent data suggest that molecular manifestations of the ageing process also hallmark the pathogenesis of chronic lung diseases, which may impact pharmacology and toxicology., Recent Findings: In particular, absorption, distribution, metabolism and excretion (ADME) processes of drugs and toxins alter because of ageing. Polypharmacy, which is quite usual with increasing age, increases the risk of drug-drug interactions. Individual differences in combination of drugs use in conjunction with individual variations in drug metabolizing enzymes can influence lung function., Summary: Exploring exposure throughout life (i.e. during ageing) to potential triggers, including polypharmacy, may avoid lung disease or unexplained cases of lung damage. Understanding of the ageing process further unravels critical features of chronic lung disease and helps to define new protective targets and therapies. Optimizing resilience can be key in pharmacology and toxicology and helps in maintaining healthy lungs for a longer period., (Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose of Review: Critical review on the notion that exposure to pesticides and herbicides lead to adverse effects in pulmonary health., Recent Findings: The lung effects of several chemical classes of pesticides and herbicides is biologically plausible. However, the studies that describe the association between exposure and toxic lung effects have numerous limitations. Critical evaluation of the studies that are performed shows that assessment of occupational or environmental exposure to pesticides and herbicides is cumbersome. Moreover, the health effects are not always clearly established due to the use of questionnaires and self-reported data instead of lung function measurements or diagnostic work-up by physicians.Future studies should preferably better characterize the exposure. Genetic phenotyping should be included to understand and strengthen possible (individual) associations between exposure and health outcome. It should be realized that combined exposure to multiple environmental chemicals may lead to different health effects than exposure to individual chemicals., Summary: The relation between exposure to pesticides and herbicides and lung toxicity is less clear than generally assumed. Adverse lung effects seem multifactorial and needs further research. Preventive measures remain key., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose of Review: The current review focuses on serious pulmonary toxicity after inhalation of over the counter available pyrethroid-based insecticides. Pyrethroid is a synthetic product of pyrethrin, which in turn is the active ingredient of pyrethrum, a flower extract., Recent Findings: On the contrary, a large gap of knowledge exists in the association of interstitial lung disease (ILD) with pyrethroids. So far, two cases of ILD, one associated with pyrethrin and one associated with pyrethrum, were described. Existing literature on both other (pulmo)toxic effects of pyrethroids in human and animals is summarized., Summary: We present three cases of severe pulmonary toxicity after inhalation of pyrethroid-based insecticides demanding hospitalization and oxygen therapy. One of these cases died. Although a causal relationship was hard to establish, these cases all demonstrated an obvious history of (repeated) pyrethroid exposure associated with ILD. Moreover, other causes of ILD as well as infections were excluded. Furthermore, studies in mammals as well as aquatic animals confirm (pulmonary) toxicity of pyrethroids. The occurrence of toxicity is dose-dependent but also associated with individual susceptibility. Therefore, we would like to acknowledge that awareness of potential hazards of commercially available insecticides containing pyrethroids to both medical physicians and the public is mandatory., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose of Review: The aim of this article is to describe the known health disparities that exist among patients with sarcoidosis by socioeconomic status, race, and gender, review potential contributors to health disparities in sarcoidosis, investigate the intersectionality among socioeconomic status, race, and gender in sarcoidosis, and outline a research agenda to address these disparities., Recent Findings: Recent studies have reported the significant financial strain a diagnosis of sarcoidosis has on individuals and the disproportionate affect the strain has on low socioeconomic status individuals, Blacks, and females. Worse dyspnea, lower health-related quality of life, and higher rates of mortality and hospitalization are more common among those who are Black, female, or of low socioeconomic status., Summary: Health disparities in sarcoidosis by socioeconomic status, race, and gender have been described for decades. In this review, we describe potential contributors to health disparities including stress and propose interventions to address disparities including creating educational programs accessible for low-income patients and caregivers, targeting medication adherence and trust in physicians and the medical system, and ensuring access to high-quality care for all patients. As clinicians and researchers, we owe it to our patients to not only describe the health disparities that exist but also stimulate to achieve improvement in sarcoidosis.

Purpose of Review: Idiopathic pulmonary fibrosis (IPF) is a terminal lung disease of largely unknown cause. Gastroesophageal reflux disease (GERD) was recently discovered to be a trigger for the development of IPF. The current pharmaceutical approach to IPF falls short and there is a pressing need for improved therapeutic options. The present review describes the currently available knowledge regarding the role of oxidative stress and inflammation in the pathophysiology of IPF and GERD and determines the potential use of antioxidants as a treatment option for GERD-associated IPF., Recent Findings: IPF and GERD share a similar pathophysiology, as oxidative stress and inflammation play a pivotal role in both conditions. This raises the question whether antioxidant treatment could be a well-tolerated and effective means to alleviate at least some of the symptoms of both conditions. In IPF, antioxidant supplementation complements the inadequately working antioxidant defense system of the lung, reducing oxidative stress and inflammation. In GERD, antioxidants increase levels of endogenous antioxidants, decrease pepsin and gastric acid production, lipid peroxidation, and ulceration, and alleviate subsequent damage to the gastric mucosa., Summary: The increased comorbidity of GERD in IPF patients makes it clear that there is a connection between GERD and IPF. As current treatment options are still inadequate to improve the condition and increase the survival rate of IPF patients, alternative treatment options are crucial. Based on the reviewed scientific evidence, antioxidant supplementation could complement standard IPF treatment, certainly in GERD-associated IPF.

Purpose of Review: The diagnosis of drug-induced interstitial lung disease (DI-ILD) is challenging and mainly made by exclusion of other possible causes. Toxicity can occur as a cause of drug(s) or drug-drug interactions. In this review, we summarize the possible role of pharmacogenetics of metabolizing enzymes in DI-ILD., Recent Findings: Knowledge of the genetic predispositions of enzymes involved in drug metabolization and their relation with proposed cytotoxic mechanisms of DI-ILD, in particular direct cell toxicity and free oxygen radical production is increasing. The cytochrome P450 enzyme family and other enzymes play an important role in the metabolism of all sorts of ingested, injected, or inhaled xenobiotic substances. The liver is the major site for metabolism. Metabolic cytotoxic mechanisms have however also been detected in lung tissue. Polymorphisms in genes coding for enzymes that influence metabolic activity may lead to localized (toxic) reactions and tissue damage. This knowledge may be helpful in preventing the risk of DI-ILD., Summary: Drug toxicity can be the consequence of absence or very poor enzyme activity, especially if no other metabolic route is available. In the case of reduced enzyme activity, it is recommended to reduce the dose or to prescribe an alternative drug, which is metabolized by a different, unaffected enzyme system to prevent toxic side effects. However, enhanced enzyme activity may lead to excessive formation of toxic and sometimes reactive metabolites. Therefore, knowing a patient's drug-metabolizing profile before drug prescription is a promising way to prevent or explain DI-ILD.

Purpose of Review: Sarcoidosis is a complex disease with many faces, and the clinical manifestation and course of neurosarcoidosis are particularly variable. Although neurosarcoidosis occurs in up to 10% of sarcoidosis patients, it can lead to significant morbidity and some mortality., Recent Findings: Three criteria are usually required for a diagnosis of (neuro)sarcoidosis: clinical and radiologic manifestations, noncaseating granulomas, and no evidence of alternative disease. Recent guidelines have helped to clarify criteria for diagnosing neurosarcoidosis. No firm guidelines exist on whether, when, and how treatment should be started. Treatment depends on the presentation and distribution, extensiveness, and severity of neurosarcoidosis. As regards evidence-based treatment, only a few randomized controlled trials have been done. Hence, several aspects of (neuro)sarcoidosis management are not fully addressed by the current literature., Summary: Significant advances have been made in the potential and accuracy of diagnostics for neurosarcoidosis. Treatment should be approached within the context of the patient's anticipated clinical course, avoidance of adverse drug effects, and, if necessary, from the perspective of the comprehensive management of a chronic disease. A multidisciplinary approach to the management of sarcoidosis is strongly recommended.

Purpose of Review: Fatigue is a problem experienced by many patients suffering from chronic diseases, including sarcoidosis patients. It has a substantial influence on patients' quality of life (QoL). It is, therefore, important to properly assess fatigue with a valid and reliable measure. The Fatigue Assessment Scale (FAS) is the only validated self-reporting instrument classifying fatigue in sarcoidosis. The aim of this review was to examine the psychometric properties of the FAS and the diseases and languages in which it has been used. Studies among sarcoidosis patients were also reviewed in terms of outcomes., Recent Findings: Studies were identified by searching the electronic bibliographic database Pubmed. Search terms used were: FAS and fatigue. Articles were included in the review if the FAS had been used to assess fatigue., Results: Since its introduction, the FAS was used in 26 different diseases or conditions, including stroke, neurologic disorders, rheumatoid arthritis, idiopathic pulmonary fibrosis and sarcoidosis. Its reliability and validity have proved to be good. Unidimensionality has been established. So far, the FAS is available in 20 languages and widely used in sarcoidosis. Digital versions as well as PDFs of various languages are available online (www.wasog.org)., Summary: The FAS has good psychometric qualities for the diseases in which it has been examined, including sarcoidosis, and can be used in clinical practice. Healthcare workers can use the FAS to assess fatigue in the management, follow-up and clinical care programmes for their patients consistently across countries, as well as in clinical research.

Purpose of Review: Sarcoidosis is a chronic disease, which is routinely treated with corticosteroids. Steroid resistance or steroid-induced adverse effects require alternatives. Other immune-modulating pharmacological treatments have been developed, and therefore expanded tremendously. Until now, the role of nutrition in the overall management of sarcoidosis has been neglected although anti-inflammatory properties of nutritional components have been known for many years now. New nutritional possibilities emerge from already existing data and offer new therapeutic avenues in the treatment of sarcoidosis., Recent Findings: Various dietary components have been shown to reduce pulmonary inflammatory processes. It is increasingly recognized, however, that the specificity and magnitude of the effect of nutrition differs from pharmacological interventions. Conventional randomized clinical trials are less suitable to test the effect of nutrition in comparison with testing drugs. Mechanistic knowledge on the action of dietary components in conjunction with an increasing understanding of the molecular processes underlying steroid resistance (as investigated in asthma and COPD and unfortunately hardly in sarcoidosis) lead to exciting suggestions on combinations of nutrition/nutritional bioactive compounds and corticosteroids that may benefit sarcoidosis patients., Summary: In order to understand the effects of nutrition in chronic disease, it is important to elucidate mechanisms and pathways of effects. Several complementing lines of evidence should be integrated in order to be able to advise sarcoidosis patients on a healthy diet as such or in combination with prescribed anti-inflammatory therapy.

Purpose of Review: Fibrosing interstitial pneumonias are associated with various stages of fibrosis. The cause of this group of syndromes remains largely unknown. For most of these diseases, a genetic basis, environmental factors and certain triggers have been suggested as possible risk factors. Various studies have found an association between genetic polymorphisms, or the presence of certain variant alleles, and the occurrence and/or progression of interstitial pneumonias of unknown origin. An acute exacerbation of idiopathic pulmonary fibrosis shows characteristics of diffuse alveolar haemorrhage (DAH). DAH can be aggravated by vitamin K deficiency. This review deals with pharmacogenetic factors underlying interindividual differences of vitamin K status in patients with interstitial pneumonias and the possibilities for a personalized approach to patient management., Recent Findings: DAH has been associated with the presence of variant alleles in vitamin K epoxide reductase complex 1, cytochrome P450 (CYP)2C9 and CYP2C19 genes. Vitamin K deficiency has been associated with an increased risk for the development of DAH and progression and/or deterioration of interstitial pneumonias. This is in line with plausible pathophysiological mechanisms. However, clinical use should be confirmed., Summary: DAH has been associated with vitamin K deficiency and suggested as potential trigger of fibrosing interstitial pneumonias. Information on genetic variation might benefit ongoing/new clinical trials, design of which should reflect needs to address relevance of testing gene variants. Whether vitamin K supplementation may prevent exacerbations or progression of interstitial pneumonias needs to be explored in future studies.

Purpose of Review: Neurosarcoidosis occurs in 5% of patients with sarcoidosis and can be difficult to diagnose. In this review we discuss the most recent advances in our understanding of the disease, describing clinical characteristics, diagnostic process, treatment, and prognosis., Recent Findings: Clinical presentation is heterogeneous with most patients presenting with cranial nerve palsy, headache, or sensory abnormalities. Patients are classified according to probability of the diagnosis with the Zajicek criteria. In these criteria, histopathological confirmation of noncaseating granulomas in affected tissue outside the nervous system is key. Radiological abnormalities on neuroimaging are nonspecific. No biomarkers have been described that adequately identify patients with sarcoidosis. However, soluble interleukin-2 receptor is a relatively novel biomarker that may be useful. In addition to HRCT scan, F-FDG PET-CT scanning can identify occult locations of disease activity and aid in obtaining pathological confirmation. Despite the use of new therapies, still a third of patients remains stable, deteriorate, or die., Summary: Diagnosing and treating patients with neurosarcoidosis remains a challenge. Long-term prospective studies evaluating patients suspected of neurosarcoidosis are needed to assess sensitivity and specificity of ancillary investigations and diagnostic criteria. Furthermore, future studies are needed to evaluate the prognosis and the optimal treatment strategy.

Purpose of Review: To discuss cause, clinical manifestations, diagnostics, and treatment of small fiber neuropathy (SFN). The diagnosis is difficult and can be easily missed., Recent Findings: SFN causes high morbidity with disabling symptoms and impact on quality of life. Patients may benefit from being diagnosed with SFN, even if no underlying cause is identified and no specific treatment is yet available. Recently, genetic mutations as a possible cause of SFN were identified. Clinical diagnostic criteria have been proposed, but no gold standard exists, and each test has its limitations. The diagnosis requires a combination of typical symptoms, abnormal neurologic findings, and absence of large fiber involvement. Clinicians should be aware of overlapping symptoms of SFN and fibromyalgia. Treatment is often difficult, even when the underlying cause is identified and appropriately treated. Usually, only symptomatic relief of complaints is available., Summary: Awareness of SFN and related symptoms is of great clinical relevance. Guidelines for appropriate diagnostic workup using a stepwise approach involving a combination of tests are warranted. Even if no treatment is available, patients may benefit from timely recognition of SFN.

Purpose of Review: Sarcoidosis is a granulomatous disease which affects multiple organs. Its therapeutic management is very challenging due to the heterogeneity in disease manifestation and clinical course, as well as the potential side effects of the immunosuppressive therapy. An overview of presently available second-line and third-line systemic agents is provided., Recent Findings: Because curative treatment is currently not available for sarcoidosis, nonspecific immunosuppression with prednisone remains the first-choice therapy. However, as chronic use of corticosteroids is accompanied with severe adverse events, timely implementation of appropriate steroid-sparing cytotoxic agents is important. Commonly prescribed second-line agents in sarcoidosis are methotrexate, azathioprine, leflunomide and hydroxychloroquine. Nevertheless, the evidence supporting their use is limited. Third-line treatment options, including tumor necrosis factor-alpha inhibitors infliximab and adalimumab and the experimental therapeutic rituximab, are currently reserved for patients refractory to standard therapy., Summary: A better insight into the advantages and disadvantages of second-line and third-line treatment is important. The long-term effects of immunosuppressive agents, the optimal starting and maintenance dosages, and the best interval and discontinuation regimens should be elucidated. Identified associations of polymorphisms with treatment response suggest a step towards personalized medicine. Future research should focus on the role for pharmacogenetic and phenotypic predictors of treatment response and toxicity.

Purpose of Review: It is important to gain knowledge and understanding about the appropriate use of PET scan in the management of sarcoidosis patients. This means that, in view of the radiation dose and costs, defining appropriate indications for PET scanning in sarcoidosis patients is vital., Recent Findings: PET has been shown to be a very sensitive technique for the assessment of inflammatory activity in sarcoidosis by detecting and quantifying the degree of inflammatory and granulomatous reactions that occur in the lungs and elsewhere in the body., Summary: PET is not indicated in the standard workup, but can be of great value to complement more routinely used techniques. On the basis of the current findings, PET offers added value in sarcoidosis patients with unexplained persistent disabling symptoms. PET appears especially helpful in those persistently symptomatic patients without serological signs of inflammatory activity, in patients with radiologic signs of fibrosis and in the detection of active cardiac sarcoidosis. The use of PET to assess the extent of disease can uncover a suitable location for biopsy to obtain histological evidence for the diagnosis or to explain the (mainly extrathoracic) symptoms. Furthermore, the detection of unexpected organ involvement may offer prognostic value.

Purpose of Review: This review focuses on innovations in the field of assessment and treatment of muscle weakness and exercise intolerance in sarcoidosis and the association between these and fatigue, dyspnea and quality of life (QoL)., Recent Findings: Muscle strength and exercise intolerance are prevalent in patients with sarcoidosis. Exercise testing can be used to identify the presence of strength deficits and exercise intolerance. Routinely performed clinical tests, including lung function tests and imaging methods, are only weakly related to these nonspecific health complaints. Assessment of exercise capacity might also be useful for the early detection of parenchymal involvement and diagnosis of sarcoidosis-associated pulmonary hypertension. Both muscle weakness and exercise intolerance have been suggested as underlying causes of fatigue and dyspnea complaints, resulting in reduced QoL. Research is required to find out whether a multidisciplinary rehabilitation program is of clinical benefit in the management of sarcoidosis patients., Summary: This review underlines the added value of physical testing in the management of sarcoidosis patients, especially in those with unexplained physical complaints.

Purpose of Review: Although glucocorticosteroids are considered the first-line treatment in sarcoidosis, refractory cases require alternatives, such as methotrexate (MTX). The aim of this study was to develop, on behalf of the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG), multinational evidence-based recommendations for the use of MTX in sarcoidosis for routine clinical practice., Recent Findings: A systematic literature search was conducted and combined with the opinions of sarcoidosis experts worldwide to formulate the recommendations. An online survey concerning 10 clinical questions was sent through the WASOG newsletter to sarcoidosis experts. Agreement about the recommendations amongst the world's leading sarcoidologists was evaluated. A total of 237 articles were identified, 43 of which were included. Randomized controlled trial evidence supporting the use of MTX in sarcoidosis was limited. Forty-five per cent (113 of 250) of the sarcoidosis experts contacted completed the survey (Europe 55%, North America 26% and Asia 12%). Ten recommendations were formulated concerning the indications for use, starting dose, folic acid, work-up, contraindications, monitoring, administration options in case of adverse gastrointestinal effects, hepatotoxicity, long-term safety and use during pregnancy and breast feeding., Summary: Ten multinational evidence-based recommendations for the use of MTX in sarcoidosis were developed, which are supported by the world's foremost sarcoidosis experts.

Purpose of Review: Surveillance of hepatic involvement in sarcoidosis has not been standardized. Therefore, management of hepatic involvement is a clinical challenge. This review analyses published data on the pharmacological treatment of hepatic sarcoidosis., Recent Findings: Only 5-30% of patients with hepatic sarcoidosis display symptoms. Occasionally, it has a rapid progressive course with serious complications, stressing an appropriate and carefully timed therapeutic approach. Because symptomatic hepatic sarcoidosis is uncommon, therapeutic studies are scarce. Answers to the questions when to initiate which treatment are lacking. Case reports describe beneficial effects of prednisone and the augmentation of cytotoxic and anti-tumor necrotic factor-α (TNF-α) therapy. However, because of small sample sizes, no meaningful conclusions could be drawn. In symptomatic hepatic sarcoidosis patients, it is recommended to start to treat the sarcoidosis with prednisone, preceded by ursodeoxycholic acid when signs of cholestasis are present. In refractory cases or when prednisone weaning is impossible, cytotoxic drugs or anti-TNF-α therapy should be considered., Summary: This review illustrates the importance of an appropriate therapeutic approach of sarcoidosis patients with hepatic involvement. It emphasizes the need for future studies to evaluate treatment options to avoid disease progression and hepatic complications.

Growth hormone deficiency (GHD), mostly after cranial radiotherapy (CRT), may lead to several negative effects. Young adult survivors of acute lymphoblastic leukemia (ALL) could benefit from GH therapy in different ways. Twenty ALL survivors (17.1 ± 4.3 y after diagnosis) with low bone mineral densities and/or low insulin-like growth factor-1 were included. Two of the 3 patients who only received chemotherapy had GHD. Of the 20 patients, 17 started with GH therapy and 14 completed the 2-year study period. At several time points, bone mineral density (BMD) was measured. Psychological functioning was assessed. At the start of the study, standard deviation scores of height, insulin-like growth factor-1, lumbar spine, and femoral neck BMD were all below -1. After 2 years of GH therapy, total body BMD and lean mass were significantly higher (P < 0.01 and P < 0.001, respectively), whereas the percentage fat was significantly lower (P < 0.02). Several psychological measures improved significantly after 2 years. In conclusion, GH therapy during 2 years in young adult survivors of childhood ALL did have a number of benefits, such as improvement of total body bone density and body composition. Results also suggest improvement of psychological well being. Furthermore, it also became clear that patients after chemotherapy alone should be tested for GHD.

Purpose of Review: Mercaptopurine therapy is increasingly important as immunosuppressive therapy in interstitial lung disease. We focus on human mercaptopurine metabolism and the defects in this metabolism causing adverse drug reactions., Recent Findings: Defects in mercaptopurine metabolizing enzymes like thiopurine methyltransferase and inosine triphosphate pyrophosphohydrolase lead to severe adverse drug reactions, sometimes with fatal outcome. Other enzymes, still not thoroughly investigated, can give rise to toxic effects or decreased efficacy in mercaptopurine therapy when the activity of these enzymes is altered., Summary: Pharmacogenetic screening of potential patients for mercaptopurine therapy is important to avoid adverse drug reactions caused by inherited enzyme deficiencies in these metabolic pathways. Pretreatment screening for deficiencies of mercaptopurine metabolizing enzymes will significantly reduce the number of patients with an adverse drug reaction and concomitantly associated healthcare costs.

Purpose of Review: The aim of this review is to provide information on the influence of interstitial lung disease on patients' health status and quality of life, with an emphasis on which aspects might be affected by interstitial lung disease., Recent Findings: The quality of life of sarcoidosis patients with current symptoms and patients with idiopathic pulmonary fibrosis is mainly impaired in the domains of physical health and level of independence. Concerning health status, sarcoidosis patients report reduced physical functioning, especially when they have current symptoms. The health status of idiopathic pulmonary fibrosis patients is impaired in almost all aspects. Subjectively assessed dyspnea is related to health status. A relationship between treatment and health status has not been found., Summary: Results from the existing studies show that both illnesses have a substantial impact on patients' life. Studies aiming at measuring quality of life or health status in other interstitial lung diseases are scarce or nonexistent.

Idiopathic interstitial pneumonia is a heterogeneous group of diseases. Recently, the importance of a detailed differentiation of nonspecific interstitial pneumonia from other idiopathic interstitial pneumonias has been demonstrated. Most critical appeared to be a proper classification based on clinical presentation, high-resolution CT findings, bronchoalveolar lavage fluid cell findings, and histopathology. This classification may guide the most appropriate therapeutic approach and has significant implications regarding prognosis. Recent advances in the diagnosis and management of nonspecific interstitial pneumonia, cellular and fibrosing variants, are discussed.