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Year : 2019  |  Volume : 14  |  Issue : 4  |  Page : 259-260

Pulmonary sarcoidosis – Predictors of relapse: Are we there yet?

Department of Clinical Immunology and Rheumatology, St Johns Medical College Hospital, Bengaluru, Karnataka, India

Date of Web Publication31-Dec-2019

Correspondence Address:
Dr. Vineeta Shobha
Department of Clinical Immunology and Rheumatology, St Johns Medical College Hospital, Bengaluru - 560 034, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_168_19

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How to cite this article:
Shobha V. Pulmonary sarcoidosis – Predictors of relapse: Are we there yet?. Indian J Rheumatol 2019;14:259-60

How to cite this URL:
Shobha V. Pulmonary sarcoidosis – Predictors of relapse: Are we there yet?. Indian J Rheumatol [serial online] 2019 [cited 2021 Oct 15];14:259-60. Available from:

Humankind has walked on the moon, is set to land on Mars in the near future, but sadly, has not solved the enigma of sarcoidosis. This disease, sarcoidosis, has baffled clinicians, researchers, and scientists alike. Heterogeneous clinical presentations, unclear pathophysiology, and inability to pinpoint etiologic agent have halted progress in its therapeutics and research. So much so that there is a paucity of clear-cut classification or diagnostic criteria for sarcoidosis. The American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and Other Granulomatous[1] statement on sarcoidosis was published in 1999. It suggests that “when clinico-radiological findings are supported by histologic evidence of noncaseating epithelioid cell granulomas,” the diagnosis of sarcoidosis can be entertained. However, the clinicoradiological findings are not clearly described. Subsequently, Heinle and Chang[2] have proposed a diagnostic criterion based on a combination of clinical, radiographic, and histological features alongside the elimination of disorders with similar clinical features. Bickett et al.[3] have tried to quantitate the clinical features consistent with biopsy-proven sarcoidosis to enhance diagnostic certainty.

The natural history of sarcoidosis is difficult to predict. There are no clear-cut assessment tools to assess response to treatment, define remission, and predict outcome. The ACCESS study[4] has demonstrated that the extent of organ involvement at presentation determines prognosis and outcome, as less than quarter of patients develop new organ involvement on follow-up in the next 2 years. Many laboratory parameters have been evaluated to determine response to therapy and predict outcome, but none have acceptable sensitivity or specificity. Currently, the best predictor of outcome seems to be through longitudinal observation of symptoms and signs in affected organ systems.

In the current edition of IJR, Nath et al.[5] report their vast experience of pulmonary sarcoidosis and attempt to determine factors predicting relapse. In this retrospective observational study, histopathologic demonstration of noncaseating granuloma along with compatible clinical picture was considered. Most patients (117/120) in this cohort received glucocorticoid therapy in view of their clinical condition, while almost half of patients also received additional immunosuppressant. Almost a quarter of patients relapsed after discontinuation of corticosteroid therapy after a median of 4 months. Univariate analysis revealed that increased duration of symptoms, history of fatigue, aspartate transaminase >40, alanine transaminase >40 at presentation, and higher Scadding stage were significant factors predicting relapse. However, on multivariate analysis, none of these factors except fatigue seemed to have significant value. In this study, relapse was defined recurrence of significant symptoms along with radiological worsening in the form of an increase in the size of lymph nodes or appearance of parenchymal nodules or increase in serum angiotensin-converting enzyme, serum calcium, and 24-h urinary calcium. Authors have commented that persistent subclinical disease activity may have been misinterpreted as relapse, thereby implying that unless a specific biomarker or outcome measure is identified, this clinical imprecision will persist. Soluble interleukin-2 receptor and 18F-fluorodeoxyglucose positron-emission tomography scan have been investigated as markers for remission and residual disease, but with inconsistent results.

It is noteworthy that, even though extrapulmonary manifestations were noted in a quarter, no musculoskeletal manifestations were noted. In a recent multicenter retrospective case series[6] from India involving 11 rheumatology centers, 117 patients of sarcoid arthropathy have been described. Those with oligoarticular disease had responded better to treatment than those with polyarticular disease.

It appears that clinical studies alone are not likely to provide the solution to many unanswered queries in diagnosis, management, and prognostication of this inscrutable disease. Biosignatures based on proteomics, metabolomics, gene expression profiling, and micro-RNA expression may help break the enigma of this mysterious disease. This is an unexplored area for research from institutes involved in basic medical science or biotechnological research in India.

Time has come to streamline and rationalize the current arbitrary approach to the diagnosis of sarcoidosis, clearly define relapse, remission, and outcome measures, as has been done for many immunomediated inflammatory diseases such as lupus and inflammatory myositis. A multi-institution consensus approach would probably be the best way forward.

  References Top

Hunninghake GW, Costabel U, Ando M, Baughman R, Cordier JF, du Bois R, et al. ATS/ERS/WASOG statement on sarcoidosis. American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and other Granulomatous Disorders. Sarcoidosis Vasc Diffuse Lung Dis 1999;16:149-73.  Back to cited text no. 1
Heinle R, Chang C. Diagnostic criteria for sarcoidosis. Autoimmun Rev 2014;13:383-7.  Back to cited text no. 2
Bickett AN, Lower EE, Baughman RP. Sarcoidosis diagnostic score: A systematic evaluation to enhance the diagnosis of sarcoidosis. Chest 2018;154:1052-60.  Back to cited text no. 3
Judson MA, Baughman RP, Thompson BW, Teirstein AS, Terrin ML, Rossman MD, et al. Two year prognosis of sarcoidosis: The ACCESS experience. Sarcoidosis Vasc Diffuse Lung Dis 2003;20:204-11.  Back to cited text no. 4
Nath A, Hashim Z, Khan A, Gupta M, Neyaz Z, Misra DP, et al. Experience of sarcoidosis and factors predicting relapse at a tertiary care institute in North India. DOI:10.4103/injr.injr_102_19.  Back to cited text no. 5
Arthritis in Sarcoidosis Group (ASG), Agarwal V, Agrawal V, Aggarwal A, Aggarwal P, Chowdhury AC, et al. Arthritis in sarcoidosis: A multicentric study from India. Int J Rheum Dis 2018;21:1728-33.  Back to cited text no. 6


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