Importance
Sarcoidosis is an inflammatory granulomatous disease of unknown cause that affects an estimated 2 to 160 people per 100 000 worldwide and can involve virtually any organ. Approximately 10% to 30% of patients with sarcoidosis develop progressive pulmonary disease.


Observation
Among patients with pulmonary sarcoidosis, the rate of spontaneous remission without serious sequelae ranges from 10% to 82%. However, lung disease progression occurs in more than 10% of patients and can result in fibrocystic architectural distortion of the lung, which is associated with a mortality rate of 12% to 18% within 5 years. Overall, the mortality rate for sarcoidosis is approximately 7% within a 5-year follow-up period. Worldwide, more than 60% of deaths from sarcoidosis are due to pulmonary involvement; however, more than 70% of deaths from sarcoidosis are due to cardiac involvement in Japan. Up to 70% of patients with advanced pulmonary sarcoidosis develop precapillary pulmonary hypertension, which is associated with a 5-year mortality rate of approximately 40%. Patients with sarcoidosis and precapillary pulmonary hypertension should be treated with therapies such as phosphodiesterase inhibitors and prostacyclin analogues. Although optimal doses of oral glucocorticoids for pulmonary sarcoidosis are unknown, oral prednisone typically starting at a dose of 20 mg/d to 40 mg/d for 2 to 6 weeks is recommended for patients who are symptomatic (cough, dyspnea, and chest pain) and have parenchymal infiltrates and abnormal pulmonary function test results. Oral glucocorticoids can be tapered over 6 to 18 months if symptoms, pulmonary function test results, and radiographs improve. Prolonged use of oral glucocorticoids may be required to control symptoms and stabilize disease. Patients without adequate improvement while receiving a dose of prednisone of 10 mg/d or greater or those with adverse effects due to glucocorticoids may be prescribed immunosuppressive agents, such as methotrexate, azathioprine, or an anti-tumor necrosis factor medication, either alone or with glucocorticoids combined with appropriate microbial prophylaxis for Pneumocystis jiroveci and herpes zoster. Effective treatments are not available for advanced fibrocystic pulmonary disease.


Conclusions and Relevance
Sarcoidosis has a mortality rate of approximately 7% within a 5-year follow-up period. More than 10% of patients with pulmonary sarcoidosis develop progressive disease and more than 60% of deaths are due to advanced pulmonary sarcoidosis. Oral glucocorticoids with or without another immunosuppressive agent are the first-line therapy for symptomatic patients with abnormal pulmonary function test results and lung infiltrates. Patients with sarcoidosis and precapillary pulmonary hypertension should be treated with therapies such as phosphodiesterase inhibitors and prostacyclin analogues.

Diagnosis and Treatment of Pulmonary Sarcoidosis: A Review.

Interstitial lung diseases

Sarcoidosis is a multisystem granulomatous disease, associated with significant morbidity and impaired quality of life. Treatment is aimed at recovering organ function, reducing symptom burden and improving quality of life. Because of the heterogeneity and variable disease course, a comprehensive, multidisciplinary approach to care is needed. Comprehensive care includes not only pharmacological interventions, but also supportive measures aimed at relieving symptoms and improving quality of life. The purpose of this review is to summarize the most recent knowledge regarding different aspects of care and propose a structured approach to sarcoidosis management.

/pdf/comprehensive-care-for-patients-with-sarcoidosis-3ktkvckpw2.pdf

Comprehensive Care for Patients with Sarcoidosis

Interstitielle Lungenparenchymerkrankungen (ILD) stellen eine heterogene Gruppe chronischer Lungenerkrankungen unterschiedlicher Ätiologie dar, die neben dem Interstitium auch den Alveolarraum und das Bronchialsystem betreffen können. Unterschieden werden idiopathische Formen, wie die idiopathische Lungenfibrose, von Formen mit bekannter Ursache (beispielsweise medikamenten- oder kollagenoseassoziierte sowie granulomatöse ILD). Gemäß der Global Burden of Disease Study zeigte sich in den letzten Jahrzehnten ein deutlicher Anstieg der Inzidenz, und in den nächsten 20 Jahren ist mit einer Verdopplung ILD-assoziierter Todesfälle zu rechnen. Therapeutisch werden prädominant „inflammatorische“ Formen immunmodulatorisch behandelt, prädominant fibrosierende Formen zumindest bei Progress antifibrotisch, in manchen Fällen werden die beiden Behandlungsstrategien kombiniert. Die Differenzialdiagnostik ist komplex, aber therapieentscheidend, sodass der multidisziplinären Falldiskussion eine relevante Bedeutung zukommt. 

Sarcoidosis, an inflammatory multi-organ disease with a wide variety of clinical manifestations, affecting people of working age. Patients suffer from a broad spectrum of physical symptoms of varying severity that impact function including cognitive impairment and disabling fatigue. The Dutch Sarcoidosis Society identified a knowledge gap in various facets related to work ability. The aim of this study was to assess sarcoidosis patients’ perceived problems related to work performance, employer, and disability evaluations. A cross-sectional web-based anonymous survey was conducted among Dutch sarcoidosis patients recruited through sarcoidosis patient societies and outpatient sarcoidosis clinics. This investigation queried work performance, employer support, and disability evaluations. The study sample included 755 patients of whom 43% (n = 328) had undergone disability evaluation and were significantly more likely to experience extrapulmonary symptoms, severe fatigue, reduced exercise capacity along with memory problems and concentration problems with higher mean FAS and SFNSL-scores. Of these 328, 37% (n = 121) perceived they had not been listened to or taken seriously at assessments, and 38% (n = 124) disagreed with the outcome of disability assessments by benefits authorities; 75% (n = 93) appealed or requested re-assessment. A better understanding of sarcoidosis-related impact on work ability and quantification of disease burden is needed. Education for medical examiners and employers on sarcoidosis may improve quality of assessments and work accommodations. Development of guidelines for benefit authorities, which consider the broad impact of sarcoidosis beyond that of reduced pulmonary function, including extra-pulmonary assessment like fatigue, cognitive difficulties, as well as other organ involvement are needed.

/pdf/sarcoidosis-and-work-participation-the-need-to-develop-a-4wrktrd2we.pdf

Sarcoidosis and Work Participation: The Need to Develop a Disease-Specific Core Set for Assessment of Work Ability

Background The major reasons to treat sarcoidosis are to lower the morbidity and mortality risk or to improve quality of life (QoL). The indication for treatment varies depending on which manifestation is the cause of symptoms: lungs, heart, brain, skin, or other manifestations. While glucocorticoids (GC) remain the first choice for initial treatment of symptomatic disease, prolonged use is associated with significant toxicity. GC-sparing alternatives are available. The presented treatment guideline aims to provide guidance to physicians treating the very heterogenous sarcoidosis manifestations. Materials and Methods A European Respiratory Society Task Force (TF) committee composed of clinicians, methodologists, and patients with experience in sarcoidosis developed recommendations based on the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology. The committee developed eight PICO (Patients, Intervention, Comparison, Outcomes) questions and these were used to make specific evidence-based recommendations. Results The TF committee delivered twelve recommendations for seven PICOs. These included treatment of pulmonary, cutaneous, cardiac, and neurologic disease as well as fatigue. One PICO question regarding small fiber neuropathy had insufficient evidence to support a recommendation. In addition to the recommendations, the committee provided information on how they use alternative treatments, when there was insufficient evidence to support a recommendation. Conclusions There are many treatments available to treat sarcoidosis. Given the diverse nature of the disease, treatment decisions require an assessment of organ involvement, risk for significant morbidity, and impact on QoL of the disease and treatment. Message An evidence based guideline for treatment of sarcoidosis is presented. The panel used the GRADE approach and specific recommendations are made. A major factor in treating patients is the risk of loss of organ function or impairment of quality of life.

https://erj.ersjournals.com/content/erj/early/2021/06/10/13993003.04079-2020.full.pdf

ERS clinical practice guidelines on treatment of sarcoidosis

Sarcoidosis patient survey reveals QoL and functionality are required as core outcomes in treatment and care, along with more multidisciplinary working by clinicians and the establishment of specialist sarcoidosis centres in every European country http://ow.ly/DTvt30mQnqc.

https://openres.ersjournals.com/content/erjor/4/4/00141-2018.full.pdf

Sarcoidosis: patient treatment priorities

Purpose of Study: A sarcoidosis treatment guidleine is being developed by a European Respiratory Society (ERS) working group. The sarcoidosis support groups were asked to provide input regarding what outcomes were most important to the patient. Methods: Sarcoidosis support groups in collaboration with the European Lung Foundation developed an outcome survey that was translated into six languages. Individual outcomes were given overall titles and defintiions for each outcome were provided. Patients were asked to rank each outcome on a 5 point scale from 1 (extremely important) to 5 (not important at all). Results: To date, 778 individuals have completed the survey in their native langauge: 270 German, 158 English, 147 Italians. 112 Dutch, 73 Spanish, and 8 French using an online survey. The eight outcomes and scores were: quality of life (1.41); Functionality (1.43); Survival (1.69); Imaging (1.84); Adverse events (1.86); Blood tests (1.98); Pulmonary function tests (2.06). All outcomes ranged from Extremely important (1) to Very important (2). Of the outcomes, quality of life and functionality were the most important, while blood tests and pulmonary function tests were less important outcomes. Imaging was ranked as a more important outcome than blood or pulmonary function tests. Conclusion: Sarcoidosis patients ranked treatments which improved quality of life and functionality as most important outcomes. Patients ranked adverse events as a less important outcome than quality of life, survival, and functionality.

Bernd Quadder

Papers

ERS clinical practice guidelines on treatment of sarcoidosis

Sarcoidosis: patient treatment priorities

Sarcoidosis treatment and outcomes: what is most important to patients?