Consequences of Sarcoidosis

Exercise intolerance, muscle weakness, dyspnoea, and fatigue are frequent complications in symptomatic sarcoidosis patients. Pulmonary rehabilitation improves exercise capacity, symptoms, and quality of life in patients with chronic respiratory diseases. Our objective was to systematically determine the effects of pulmonary rehabilitation in patients with sarcoidosis.

A systematic review was conducted in seven databases. Studies that applied pulmonary rehabilitation in patients with sarcoidosis were reviewed. Two independent reviewers analysed the studies, extracted the data and assessed the quality of evidence.

Of the 406 reports returned by the initial search, five articles reporting on 184 patients were included in the data synthesis. Two studies included multi-component exercise, one inspiratory muscle training, one a physical activity incentivisation programme, and one a telerehabilitation program. In the intervention group (IG), we found significant improvement in exercise capacity (SMD 1.65, 95%CI 0.45, 2.86 points, p = 0.006). If we only analyse the studies that performed the 6-min walking test, the IG walked 40.3 (CI95% 20.3, 60.2) m higher than the control group (CG) (p < 0.001). Additionally, dyspnoea score was reduced (MD -0.42 95%CI -0.75, −0.10, p = 0.002). However, fatigue, quality of life and pulmonary function did not show any change.

Pulmonary rehabilitation could improve exercise capacity and dyspnoea perception in patients with sarcoidosis.

Besides dyspnoea and cough, patients with idiopathic pulmonary fibrosis (IPF) or sarcoidosis may experience distressing non-respiratory symptoms, such as fatigue or muscle weakness. However, whether and to what extent symptom burden differs between patients with IPF or sarcoidosis and individuals without respiratory disease remains currently unknown.

To study the respiratory and non-respiratory burden of multiple symptoms in patients with IPF or sarcoidosis and to compare the symptom burden with individuals without impaired spirometric values, FVC and FEV1 (controls).

Demographics and symptoms were assessed in 59 patients with IPF, 60 patients with sarcoidosis and 118 controls (age ≥18 years). Patients with either condition were matched to controls by sex and age. Severity of 14 symptoms was assessed using a Visual Analogue Scale.

44 patients with IPF (77.3% male; age 70.6±5.5 years) and 44 matched controls, and 45 patients with sarcoidosis (48.9% male; age 58.1±8.6 year) and 45 matched controls were analyzed. Patients with IPF scored higher on 11 symptoms compared to controls (p<0.05), with the largest differences for dyspnoea, cough, fatigue, muscle weakness and insomnia. Patients with sarcoidosis scored higher on all 14 symptoms (p<0.05), with the largest differences for dyspnoea, fatigue, cough, muscle weakness, insomnia, pain, itch, thirst, micturition (night, day).

Generally, respiratory and non-respiratory symptom burden is significantly higher in patients with IPF or sarcoidosis compared to controls. This emphasizes the importance of awareness for respiratory and non-respiratory symptom burden in IPF or sarcoidosis and the need for additional research to study the underlying mechanisms and subsequent interventions.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung condition marked by lung scarring that progresses over time and with usual interstitial pneumonia histology (UIP). It is linked to a worsening cough, dyspnea, and a worse quality of life. Around 3 million persons worldwide suffer from IPF, and the prevalence rises sharply with advancing age. The detection of the UIP pattern, generally using high-resolution CT; lung biopsy may be necessary in certain individuals; the diagnostic approach also includes the elimination of other interstitial lung illnesses or overlapping problems. The UIP pattern is mostly bilateral, peripheral, and basal, with clusters of subpleural cystic airspaces and reticular alterations linked to traction bronchiectasis.

Although there are still many uncertainties about how to define susceptibility, it is believed that the molecular mechanisms causing IPF reflect an abnormal reparative response to repeated alveolar epithelial damage in an aging genetically sensitive individual. With the availability of two pharmacotherapeutic drugs, pirfenidone and nintedanib, that slow physiological advancement and potentially increase progression-free survival, significant progress has been made in our knowledge of the clinical treatment of IPF. The goal of current research is to develop early biomarkers for IPF that may include circulating variables, demographic information, and imaging data.

Although stress and styles of coping with it can have a major impact on one's health and can determine the course and management of chronic diseases, no previous studies have evaluated coping strategies and their relation to emotional distress and clinical symptoms in sarcoidosis.

In two consecutive studies, we investigated differences in coping styles of sarcoidosis patients in comparison to healthy control subjects and the association of identified profiles to an objective measurement of disease (Forced Vital Capacity) and symptoms such as dyspnoea, pain, anxiety and depressive symptoms in 36 patients with sarcoidosis (study 1) and 93 patients with sarcoidosis (study 2).

Across two studies we found that patients with sarcoidosis used emotion-focused and avoidant coping significantly less often than healthy individuals, and that in both groups the profile with dominant problem (task)-focus style was the most beneficial in terms of mental health. Further, the profile of sarcoidosis patients characterized by the lowest intensity of all coping strategies was found to be superior in terms of physical health status (dyspnoe, pain and FVC level).

These findings suggest that successful management of sarcoidosis should include coping styles assessment and call for a multidisciplinary approach in diagnosis and treatment of sarcoidosis patients.

La sarcoïdose peut évoluer vers une maladie chronique dans environ 30 % des cas. Lorsqu’un traitement général est indiqué, les corticoïdes constituent le traitement de première ligne. Plus d’un tiers des patients traités par corticoïdes reçoivent un agent d’épargne cortisonique. Bien que le méthotrexate soit l’agent d’épargne le plus souvent employé, les antipaludéens de synthèse sont utilisés depuis plus de cinquante ans sur la base d’essais thérapeutiques, randomisés, de faible taille. Malgré ce faible niveau de preuve, la chloroquine ou plus souvent désormais l’hydroxychloroquine sont utilisées en pratique quotidienne, notamment pour traiter les sarcoïdoses cutanée et articulaire, ainsi que l’hypercalcémie et certaines uvéites.

Cette revue synthétise l’état des connaissances sur le traitement d’épargne cortisonique dans la sarcoïdose, notamment dans sa forme extra-pulmonaire. Ces données étayent la nécessité de conduire des essais thérapeutiques de bonne qualité pour valider l’utilisation de l’hydroxychloroquine dans cette indication spécifique.

Sarcoidosis can develop into a chronic disease in about 30% of cases. When general treatment is indicated, corticosteroids are the first-line treatment. More than one third of patients treated with corticosteroids receive a steroid-sparing agent. Although methotrexate is the most commonly used sparing agent, synthetic antimalarials have been used for more than fifty years on the basis of small, randomised, therapeutic trials. Despite this low level of evidence, chloroquine or more often hydroxychloroquine are used in daily practice, particularly to treat skin, bone and joint sarcoidosis, as well as hypercalcemia and certain types of uveitis.

This review summarises the state of knowledge on steroid-sparing therapy in sarcoidosis, particularly in its extra-pulmonary form. These data support the need for good quality therapeutic trials to validate the use of hydroxychloroquine in this specific indication.