Sarcoidosis Follow-up
- Author: Nader Kamangar, MD, FACP, FCCP, FCCM; Chief Editor: Zab Mosenifar, MD more...
Further Inpatient Care
- Monitor pulmonary function and CXR every 6-12 months.
- Assess for progression or resolution.
- Determine if previously uninvolved organs have become affected.
Further Outpatient Care
- Annual slit lamp eye examination and ECG are recommended.
Prognosis
Many patients do not require therapy, and their conditions will spontaneously improve. Markers for a poor prognosis include advanced CXR stage, extrapulmonary disease (predominantly cardiac and neurologic), and evidence of pulmonary hypertension. Multiple studies have demonstrated that the most important marker for prognosis is the initial CXR stage (see Table 2).
Table 1. Prognosis (Open Table in a new window)
| Stage | Remission (%) | Asymptomatic at 5 y (%) | CXR Clearing (%) | Mortality (%) |
| Stage I | 60-90 | 95 | 54 | 0 |
| Stage II | 40-70 | 58 | 31 | 11 |
| Stage III | 10-20 | 25 | 10 | 18 |
| Stage IV | 0 | N/A | 0 | N/A |
Although corticosteroids are used for symptom relief and remain the mainstay of therapy, their efficacy in this disease is unclear. A meta-analysis suggests that corticosteroids have little impact on sarcoidosis. However, prior studies have been hampered by their uncontrolled nature, small sample size, and the variable natural history of the disease. Since many patients' conditions improve spontaneously, showing a true benefit to therapy requires a careful control arm.
The best study addressing corticosteroids was the recently completed multicenter trial from Britain sponsored by the British Thoracic Society. In this nonrandomized study, 55 patients were selectively observed or treated with corticosteroids. Additionally, patients who were felt to have an immediate indication for steroids were treated an observed. The trial required a 6-month run-in period to exclude patients who improved spontaneously. At the end of the trial, the groups treated with long-term steroids fared better on some measures than did the patients who were observed and treated with short bursts of steroids (see the table below for greater detail).
Table 2. Results of Multicenter Trial Sponsored by the British Thoracic Society (Open Table in a new window)
| Characteristics | Group L* | Group S† | P |
| Dyspnea score (range 1-4) | 0.24 | 0.47 | NS |
| Fibrosis score (range 0-16) | 0.83 | 1.47 | NS |
| FEV1‡ (% predicted) | 95.9 | 86.9 | 0.05 |
| VC§ (% predicted) | 99.8 | 90.8 | 0.02 |
| DLCOII (% predicted) | 84.3 | 77.7 | NS |
| Weight gain (kg) | +3.26 | +0.99 | 0.02 |
| *Long-term steroids †Short bursts of steroids ‡Forced expiratory volume in 1 second §Ventilatory capacity II Diffusing capacity of lung for carbon monoxide | |||
Zabel P, Entzian P, Dalhoff K, Schlaak M. Pentoxifylline in treatment of sarcoidosis. Am J Respir Crit Care Med. May 1997;155(5):1665-9. [Medline].
Doty JD, Mazur JE, Judson MA. Treatment of sarcoidosis with infliximab. Chest. Mar 2005;127(3):1064-71. [Medline].
Yee AM, Pochapin MB. Treatment of complicated sarcoidosis with infliximab anti-tumor necrosis factor-alpha therapy. Ann Intern Med. Jul 3 2001;135(1):27-31. [Medline].
Facco M, Cabrelle A, Teramo A, Olivieri V, Gnoato M, Teolato S, et al. Sarcoidosis is a Th1/Th17 multisystem disorder. Thorax. Feb 2011;66(2):144-50. [Medline].
Swigris JJ, Olson AL, Huie TJ, et al. Sarcoidosis-related mortality in the United States from 1988 to 2007. Am J Respir Crit Care Med. Jun 1 2011;183(11):1524-30. [Medline]. [Full Text].
Okada S, Konishi N, Tsumura M, et al. Cardiac infiltration in early-onset sarcoidosis associated with a novel heterozygous mutation, G481D, in CARD15. Rheumatology (Oxford). Apr 9 2009;[Medline].
[Best Evidence] Sverrild A, Backer V, Kyvik KO, et al. Heredity in sarcoidosis: a registry-based twin study. Thorax. Oct 2008;63(10):894-6. [Medline].
Arai Y, Saul JP, Albrecht P, Hartley LH, Lilly LS, Cohen RJ. Modulation of cardiac autonomic activity during and immediately after exercise. Am J Physiol. Jan 1989;256(1 Pt 2):H132-41. [Medline].
Ardic I, Kaya MG, Yarlioglues M, et al. Impaired heart rate recovery index in patients with sarcoidosis. Chest. Jan 2011;139(1):60-8. [Medline].
Shetler K, Marcus R, Froelicher VF, et al. Heart rate recovery: validation and methodologic issues. J Am Coll Cardiol. Dec 2001;38(7):1980-7. [Medline].
Lower EE, Baughman RP. Prolonged use of methotrexate for sarcoidosis. Arch Intern Med. 1995;155:846-851. [Medline].
Baltzan M, Mehta S, Kirkham TH, Cosio MG. Randomized trial of prolonged chloroquine therapy in advanced pulmonary sarcoidosis. Am J Respir Crit Care Med. Jul 1999;160(1):192-7. [Medline].
Zic JA, Horowitz DH, Arzubiaga C, King LE Jr. Treatment of cutaneous sarcoidosis with chloroquine. Review of the literature. Arch Dermatol. Jul 1991;127(7):1034-40. [Medline].
Demeter SL. Myocardial sarcoidosis unresponsive to steroids. Treatment with cyclophosphamide. Chest. July 1988;94:202-3. [Medline].
Doty JD, Mazur JE, Judson MA. Treatment of corticosteroid-resistant neurosarcoidosis with a short-course cyclophosphamide regimen. Chest. Nov 2003;124(5):2023-6. [Medline].
Müller-Quernheim J, Kienast K, Held M, Pfeifer S, Costabel U. Treatment of chronic sarcoidosis with an azathioprine/prednisolone regimen. Eur Respir J. Nov 1999;14(5):1117-22. [Medline].
Kataria YP. Chlorambucil in sarcoidosis. Chest. Jul 1980;78(1):36-43. [Medline].
York EL, Kovithavongs T, Man SF, Rebuck AS, Sproule BJ. Cyclosporine and chronic sarcoidosis. Chest. Oct 1990;98(4):1026-9. [Medline].
Baughman RP, Judson MA, Teirstein AS, Moller DR, Lower EE. Thalidomide for chronic sarcoidosis. Chest. Jul 2002;122(1):227-32. [Medline].
Callejas-Rubio JL, Lopez-Perez L, Ortego-Centeno N. Tumor necrosis factor-alpha inhibitor treatment for sarcoidosis. Ther Clin Risk Manag. Dec 2008;4(6):1305-13. [Medline].
Bachelez H, Senet P, Cadranel J, Kaoukhov A, Dubertret L. The use of tetracyclines for the treatment of sarcoidosis. Arch Dermatol. Jan 2001;137(1):69-73. [Medline].
Nathan SD. Lung transplantation: disease-specific considerations for referral. Chest. Mar 2005;127(3):1006-16. [Medline].
Alberts C, van der Mark TW, Jansen HM. Inhaled budesonide in pulmonary sarcoidosis: a double-blind, placebo-controlled study. Dutch Study Group on Pulmonary Sarcoidosis. Eur Respir J. May 1995;8(5):682-8. [Medline].
Baughman RP, Iannuzzi MC, Lower EE, Moller DR, Balkissoon RC, Winget DB, et al. Use of fluticasone in acute symptomatic pulmonary sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. Oct 2002;19(3):198-204. [Medline].
Chesnutt AN. Enigmas in sarcoidosis. West J Med. Jun 1995;162(6):519-26. [Medline].
Devaney K, Goodman ZD, Epstein MS, Zimmerman HJ, Ishak KG. Hepatic sarcoidosis. Clinicopathologic features in 100 patients. Am J Surg Pathol. Dec 1993;17(12):1272-80. [Medline].
Gibson GJ, Prescott RJ, Muers MF, Middleton WG, Mitchell DN, Connolly CK, et al. British Thoracic Society Sarcoidosis study: effects of long term corticosteroid treatment. Thorax. Mar 1996;51(3):238-47. [Medline].
Hunninghake GW. Goal of the treatment for sarcoidosis. Minimize harm for the patient. Am J Respir Crit Care Med. Nov 1997;156(5):1369-70. [Medline].
Hunninghake GW, Gilbert S, Pueringer R, Dayton C, Floerchinger C, Helmers R, et al. Outcome of the treatment for sarcoidosis. Am J Respir Crit Care Med. Apr 1994;149(4 Pt 1):893-8. [Medline].
Johns CJ, Zachary JB, MacGregor MI, Curtis JL, Scott PP, Terry PB. The longitudinal study of chronic sarcoidosis. Trans Am Clin Climatol Assoc. 1982;94:173-81. [Medline].
Kataria YP, Holter JF. Immunology of sarcoidosis. Clin Chest Med. 1997;18:719-740. [Medline].
Moller DR. Etiology of sarcoidosis. Clin Chest Med. 1997;14:695-706. [Medline].
Newman LS, Rose CS, Maier LA. Sarcoidosis [published erratum appears in N Engl J Med 1997 Jul 10;337(2):139]. N Engl J Med. Apr 24 1997;336(17):1224-34. [Medline].
Paramothayan S, Jones PW. Corticosteroid therapy in pulmonary sarcoidosis: a systematic review. JAMA. Mar 13 2002;287(10):1301-7. [Medline].
Sharma OP. Cardiac and neurologic dysfunction in sarcoidosis. Clin Chest Med. Dec 1997;18(4):813-25. [Medline].
Winterbauer RH, Kirtland SH, Corley DE. Treatment with corticosteroids. Clin Chest Med. Dec 1997;18(4):843-51. [Medline].
Zissel G, Müller-Quernheim J. Sarcoidosis: historical perspective and immunopathogenesis (Part I). Respir Med. Feb 1998;92(2):126-39. [Medline].
| Stage | Remission (%) | Asymptomatic at 5 y (%) | CXR Clearing (%) | Mortality (%) |
| Stage I | 60-90 | 95 | 54 | 0 |
| Stage II | 40-70 | 58 | 31 | 11 |
| Stage III | 10-20 | 25 | 10 | 18 |
| Stage IV | 0 | N/A | 0 | N/A |
| Characteristics | Group L* | Group S† | P |
| Dyspnea score (range 1-4) | 0.24 | 0.47 | NS |
| Fibrosis score (range 0-16) | 0.83 | 1.47 | NS |
| FEV1‡ (% predicted) | 95.9 | 86.9 | 0.05 |
| VC§ (% predicted) | 99.8 | 90.8 | 0.02 |
| DLCOII (% predicted) | 84.3 | 77.7 | NS |
| Weight gain (kg) | +3.26 | +0.99 | 0.02 |
| *Long-term steroids †Short bursts of steroids ‡Forced expiratory volume in 1 second §Ventilatory capacity II Diffusing capacity of lung for carbon monoxide | |||

