Physical activity and training in sarcoidosis: review and experience-based recommendations

Figures & data

Table 1. Data-extraction from studies on physical training in sarcoidosis patients.

				Intervention			Outcome
Author	Year	Study design	n	Duration	Frequency	Content	Domains	Results
Huppmann et al. STROBE 20/34 [21]	2012	Retrospective cohort study	50	30 days Inpatient	4–5 days a week, 5.5 hours a day	Exercise training, breathing training, group education	Exercise capacity, lung function, QOL	Improved exercise capacity (+34 m on 6MWD); marginally improved lung function (+1% ± 0% on VC); improved SF-36 score (both physical and mental sum scores, +6 ± 1 and +10 ± 1 resp.); data regarding sarcoidosis patients obtained separately from the author
Marcellis et al. [22] STROBE 30/34	2015	Prospective cohort study	18	13 weeks Outpatient	3 times a week 1 hour	Endurance training, 50% Wmax, peripheral muscle training	Fatigue, dyspnea, QoL, exercise capacity, muscle strength	Reduced fatigue (–2.7 points; CI -4.4 to 1.1 on FAS); improved health status (WHOQOL-BREF psych. domain); improved exercise capacity (+34.6 m; CI 20.3 to 49.0 on 6MWD); m. quadriceps fem. strength improvement (+10.7 kg; CI 5.5 to 15.9)
Strookappe et al. [23] STROBE 27/34*	2015	Retrospective cohort study	90	12 weeks Outpatient	2 times a week 1 hour	Aerobic endurance training, 50% Wmax steep ramp test, peripheral muscle training	Fatigue, exercise capacity	Reduced fatigue score in training group (74% > MCID FAS); improved exercise capacity in training group (+70 m on 6MWD vs. +4 m) >10% increased 6MWD in 47% of patients vs. 5% in patients without training
Karadalli et al. [24] STROBE 25/34 PEDro 9/11	2015	Prospective, randomized, controlled, and double-blind study	30	6 weeks, outpatient, 6 sessions at home and 1 session under supervision at dept. of physiotherapy	30 min a day, 7 days a week	Inspiratory muscle training at 40% of maximal inspiratory pressure (PImax)	Exercise capacity, respiratory and peripheral muscle strength, lung function and fatigue, dyspnea, depression, and QoL	Inspiratory muscle strength improvement (PImax +45.9 cmH2O, CI 39.3 to 52.8, p < 0.001); improved exercise capacity (6MWD +66.1 m, CI 44.3 to 88.0, p < 0.001)

n: number of sarcoidosis patients; W_max: maximal wattage on cycle ergometry test (Steep Ramp Test); SRT: Steep Ramp Test; FAS: Fatigue Assessment Scale; WHOQOL-BREF: World Health Organization Quality OF Life – BREF; 6MWD: 6-minute walking distance; CI: confidence interval; FVC: forced vital capacity; MCID: minimal clinically important difference, VC: vital capacity. Results are presented as means.

Figure 1. Flow-chart of the literature review.

Table 2. Demographics of respondents to the survey [30–32].

	Europe n = 54	US and Canada n = 27	Other n = 27	Total population n = 108
Age, years
31–45/46–60/>60, %	26/54/20	41/37/22	55/30/15	36/45/19
Specialty
Pulmonologist, %	85	74	82	82
Experience, years
0–5/5–10/1015/15/25/>25, %	6/15/18/28/33	15/26/7/19/33	4/37/11/26/22	7/23/14/25/31
Percentage of patients with pulmonary sarcoidosis
Less severe (chest X-ray stage 0–I), %	37 ± 24	32 ± 25	47 ± 26	38 ± 25
Severe (chest X-ray stage II–IV), %	61 ± 25	68 ± 24	53 ± 26	61 ± 25
Percentage of patients with extrapulmonary sarcoidosis
Less severe, %	45 ± 26	41 ± 24	60 ± 31	48 ± 27
Severe, %*	54 ± 27	59 ± 23	30 ± 25	49 ± 28

*Severe: clinically relevant lesions for which treatment is recommended.

Table 3. Questions on physical training in sarcoidosis.

	Europe n = 54	US and Canada n = 27	Other n = 27	Total population n = 108
Are you familiar with physical training in sarcoidosis?
Yes/no, %	65/35	70/30	41/59	60/40
Do you consider physical training valuable in sarcoidosis?
Yes/no/not sufficiently familiar, %	89/4/7	78/0/22	67/4/30	81/3/16
Physical training is available in the region where I work for sarcoidosis patients.
Yes/no/not sufficiently familiar, %	72/20/8	67/26/7	56/33/11	67/25/8
If you do have access to physical training are you likely to refer sarcoidosis patients?
Regularly/often/always, %	67	59	67	65
Never/occasionally, %	33	41	33	35
Physical training is reimbursed by health insurers for sarcoidosis patients in my country.
Yes/sometimes/not sufficiently familiar, %	26/32/41	7/56/37	19/22/59	20/36/44
Regional restrictions have kept me from referring patients to physical training.
Yes/no/not sufficiently familiar, %	41/46/13	44/44/12	44/41/15	43/44/13

Table 4. Indications for physical training in sarcoidosis.

Do you perceive physical training to be beneficial in sarcoidosis patients with:	Europe n = 32	US and Canada n = 15	Other n = 13	Total population n = 60
Pulmonary involvement	8.2 ± 1.7	8.2 ± 1.2	7.9 ± 1.7	8.1 ± 1.6
Mild pulmonary involvement	6.7 ± 2.7	6.3 ± 3.0	6.7 ± 2.8	6.6 ± 2.7
No pulmonary involvement ^a	7.7 ± 1.9	6.8 ± 2.2	5.9 ± 2.9	7.1 ± 2.3
Fatigue	8.2 ± 1.7	7.5 ± 2.1	7.3 ± 2.8	7.8 ± 2.2
Small fiber neuropathy	6.4 ± 2.5	5.9 ± 2.7	6.8 ± 2.8	6.4 ± 2.6
Arthritis	6.2 ± 2.4	6.6 ± 2.4	5.3 ± 2.2	6.1 ± 2.4
Muscular involvement	7.3 ± 2.5	6.9 ± 2.6	7.0 ± 2.5	7.1 ± 2.5
Neurosarcoidosis	6.2 ± 2.4	5.7 ± 3.0	6.7 ± 2.4	6.2 ± 2.5
Cardiac involvement	5.7 ± 2.0	6.7 ± 2.9	5.6 ± 2.6	6.0 ± 2.4

Likert 0–10. Data are expressed as mean ± SD

p < 0.05: ^a Europe vs. Other.

Table 5. International experts’ recommendations for the use of physical training in sarcoidosis.

	Recommendation	Agreement* (mean ± SD)	LoE** and GoR**
1	The cause of fatigue in sarcoidosis patients can be multifactorial. Therefore, other causes beside sarcoidosis-associated fatigue should be excluded.	9.7 ± 0.6	2B C
2	Standardized fatigue assessment (with a validated instrument, e.g. FAS) is recommended in the work-up of sarcoidosis patients.	7.0 ± 2.4	2B B
3	In the assessment of the severity and extent of the disease in symptomatic sarcoidosis patients with fatigue and/or exercise limitation, evaluation of exercise capacity (submaximal or maximal exercise test, e.g. 6MWD, CPET) as well as muscle strength assessment should be considered.	7.2 ± 2.4	2B C
4	To achieve improvement of sarcoidosis-associated fatigue, a physical training program is recommended in symptomatic sarcoidosis patients, complementary to the medical treatment.	7.7 ± 2.9	2B C
5	Symptomatic sarcoidosis patients with fatigue and/or exercise limitation suffering from various manifestations might benefit from a supervised physical training program, not only patients with pulmonary manifestations.	7.6 ± 3.1	2B C
6	A correctly monitored supervised physical training program in sarcoidosis is safe and has no absolute contraindications based on its acceptable safety profile. However, manifestations of pulmonary hypertension and cardiac involvement should be considered as relative contraindications and caution should be exercised by the qualified supervisors.	7.7 ± 2.4	5 D
7	When starting physical training in symptomatic sarcoidosis, standardized evaluation of exercise capacity (e.g. 6MWD), muscle strength, and fatigue (e.g. FAS) is recommended at baseline and after completion of the program.	7.5 ± 2.9	3B C
8	Aerobic endurance training and peripheral muscle strengthening are both recommended in a supervised physical training program for sarcoidosis patients.	7.6 ± 2.8	3B C

LoE: Levels of Evidence; GoR: Grade of Recommendations

FAS: Fatigue Assessment Scale; 6MWD: six-minute walking distance; CPET: cardiopulmonary exercise test.

*The level of agreement was measured on a 10-point Likert scale (from 1, no agreement, to 10, full agreement).

**Level of Evidence and Grade of Recommendation according to Oxford Centre for Evidence-Based Medicine.

Huppmann P, Sczepanski B, Boensch M, et al. Effects of inpatient pulmonary rehabilitation in patients with interstitial lung disease. Eur Respir J. 2013;42:444–453.

 PubMed Web of Science ®Google Scholar

Marcellis RG, Veeke MAF, Mesters I, et al. Does physical training reduce fatigue in sarcoidosis? Sarcoidosis Vasc Diffuse Lung Dis. 2015;32:53–62.

 PubMed Web of Science ®Google Scholar

Strookappe B, Swigris J, De Vries J, et al. Benefits of physical training in Sarcoidosis. Lung. 2015;193:701–708.

 PubMed Web of Science ®Google Scholar

Karadalli MN, Bosnak-Guclu M, Camcioglu B, et al. Effects of inspiratory muscle training in subjects with sarcoidosis: a randomized controlled clinical trial. Respir Care. 2015;61:483–494.

 PubMed Web of Science ®Google Scholar

Baughman RP, Drent M, Culver DA, et al. Endpoints for clinical trials of sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2012;29:90–98.

 PubMed Web of Science ®Google Scholar

Judson MA, Costabel U, Drent M, et al. The WASOG sarcoidosis organ assessment instrument: an update of a previous clinical tool. Sarcoidosis Vasc Diffuse Lung Dis. 2014;31:19–27.

 PubMed Web of Science ®Google Scholar