Management pitfalls and prevalence of lost to follow-up due to unpredicted death in systemic sclerosis

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Patnarin Pongkulkiat
Orathai Wantha
Ajanee Mahakkanukrauh
Siraphop Suwannaroj
Chingching Foocharoen


Lost to follow-up in systemic sclerosis (SSc) is reported. Unpredicted death before visit schedule might be the result of pitfalls in patients care management. We aimed to define the prevalence of lost to follow-up due to unpredicted death in SSc and management pitfalls. A retrospective study was performed using scheduled visits to the Scleroderma Clinic, Khon Kaen University, between January 2019 and April 2020. A direct telephone call was performed when lost to follow-up was identified. If no response was received, the medical records were reviewed, and health status was requested from the Civil Registration Bureau. A total of 497 adult SSc patients with 2,040 follow-ups from 53 visits were reviewed, of whom 37 were lost to follow up due to unpredicted death for a prevalence of 7.4% (95% cumulative incidence (CI) 5.3-10.1). The respective median time from death to the next scheduled visit and median duration of disease was 19.7 days (Interquartile Range (IQR) 2.9-34) and 3.8 years (IQR 1.7-7.5). Six cases were defined as an improper interval of follow-up and monitoring that led to death before the scheduled visit. All had cardiopulmonary involvement. One case was defined as improper management given for cardiac involvement. Pneumonia and sepsis were the causes of death in 4 of 14 cases that received the immunosuppressant. Death before the scheduled follow-up visit occurred among SSc patients, particularly among those with cardiopulmonary involvement and inadequate monitoring or management. Close monitoring should be the norm for SSc patients with cardiopulmonary involvement to avoid unpredicted death.


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Pongkulkiat, P., Wantha, O., Mahakkanukrauh, A., Suwannaroj, S., & Foocharoen, C. (2022). Management pitfalls and prevalence of lost to follow-up due to unpredicted death in systemic sclerosis. Asia-Pacific Journal of Science and Technology, 27(05), APST–27.
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Silver RM. Clinical aspects of systemic sclerosis (scleroderma). Ann Rheum Dis. 1991;50 Suppl 4:854-861.

Domsic RT, Reyna RT, Lucas M, Fertig N, Medsger TA. Skin thickness progression rate: a predictor of mortality and early internal organ involvement in diffuse scleroderma. Ann Rheum Dis. 2011;70(1):104-109.

Steen VD, Medsger TA. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000;43(11):2437-2444.

Steen V. The heart in systemic sclerosis. Curr Rheumatol Rep. 2004;6(2):137-140.

Bussone G, Mouthon L. Interstitial lung disease in systemic sclerosis. Autoimmun Rev. 2011;10(5):248-255.

Cohen S. The gastrointestinal manifestations of scleroderma: pathogenesis and management. Gastroenterology. 1980;79(1):155-166.

Denton CP, Lapadula G, Mouthon L, Ladner MU. Renal complications and scleroderma renal crisis. Rheumatology. 2009;48 Suppl 3:iii32-iii35.

Liu X, Li M, Xu D, Hou Y, Wang Q, Tian Z, et al. Prevalence and clinical importance of gastroesophageal reflux in Chinese patients with systemic sclerosis. Clin Exp Rheumatol. 2012;30(2 Suppl 71):S60-S66.

Nietert PJ, Silver RM. Patterns of hospital admissions and emergency room visits among patients with scleroderma in South Carolina, USA. J Rheumatol. 2003;30(6):1238-1243.

Foocharoen C, Mahakkanukrauh A, Suwannaroj S, Nanagara R. Clinical characteristics and mortality in systemic sclerosis: a comparison between early- and late-referred diseases. J Med Assoc Thai 2014;97(1):28-35.

Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR scleroderma trials and research (EUSTAR) database. Ann Rheum Dis. 2010;69(10):1809-1815.

Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American rheumatism association diagnostic and therapeutic criteria committee. Arthritis Rheum. 1980;23(5):581-590.

Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. Classification criteria for systemic sclerosis: an ACR-EULAR collaborative initiative. Arthritis Rheum. 2013;65(11):2737-2347.

LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15(2):202-205.

Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1-13.

Savarino E, Furnari M, Bortoli N, Martinucci I, Bodini G, Ghio M, et al. Gastrointestinal involvement in systemic sclerosis. Presse Med 2014;43(10 Pt 2):e279-e291.

Young A, Namas R, Dodge C, Khanna D. Hand Impairment in Systemic Sclerosis: Various Manifestations and Currently Available Treatment. Curr Treatm Opt Rheumatol. 2016;2(3):252-269.

Cappellini MD, Motta I. Anemia in clinical practice-definition and classification: does hemoglobin change with aging? Semin Hematol. 2015;52(4):261-269.

Foocharoen C, Yenrom P, Mahakkanukrauh A, Suwannaroj S, Nanagara R. Prevalence and causes of loss to follow-up among patients with systemic sclerosis. J Clin Rheumatol. 2014;20(8):456.

Trading Economics. Thailand GDP per capita 2021 data - 2022 forecast - 1960-2020 historical - chart [Internet]. 2021 [cited 2021 Apr 26]. Available from:

Foocharoen C, Peansukwech U, Mahakkanukrauh A, Suwannaroj S, Pongkulkiat P, Khamphiw P, et al. Clinical characteristics and outcomes of 566 Thais with systemic sclerosis: a cohort study. Int J Rheum Dis. 2020;23(7):945-957.

Wolfe RM, Peacock JE. Pneumocystis pneumonia and the rheumatologist: which patients are at risk and how can PCP be prevented? Curr Rheumatol Rep. 2017;19(6):35.

Odashima K, Kagiyama N, Kanauchi T, Ishiguro T, Takayanagi N. Incidence and etiology of chronic pulmonary infections in patients with idiopathic pulmonary fibrosis. PLoS One. 2020;15(4):e0230746.

Foocharoen C, Siriphannon Y, Mahakkanukrauh A, Suwannaroj S, Nanagara R. Incidence rate and causes of infection in Thai systemic sclerosis patients. Int J Rheum Dis. 2012;15(3):277-283.

Forbes A, Marie I. Gastrointestinal complications: the most frequent internal complications of systemic sclerosis. Rheumatology. 2009;48 Suppl 3:iii36-iii39.

Sallam H, McNearney TA, Chen JDZ. Systematic review: pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis (scleroderma). Aliment Pharmacol Ther 2006;23(6):691-712.