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Diagnostic approaches and treatments of systemic sclerosis patient with interstitial lung disease

  • Fauziah Diayu Retnaningtyas ,
  • Awalia ,


Background: Systemic sclerosis or scleroderma is a rare disease with multiple complications which may include interstitial lung disease. The findings of diffuse type of systemic sclerosis, moderate restriction in pulmonary function test, interstitial pneumonia in chest x-ray, and ground glass opacity in thoracic CT-scan establish the diagnosis of interstitial lung disease associated with systemic sclerosis. We present a case of interstitial lung disease in a systemic sclerosis case, which was approached and treated comprehensively to slow the progression.

Case Presentation: A 40-year-old female was admitted to the rheumatology polyclinic of Dr. Soetomo Hospital Surabaya with persistent dry cough for two years in June 2021. The patient also complained of breathlessness, painful joints, swollen knees, and morning stiffness three months prior to admission. The positive results of antinuclear antibody and rheumatoid factor were identified in 2016, which suggested autoimmune disease of scleroderma and being treated with azathioprine 50 mg twice a day, methylprednisolone 4 mg once a day, chloroquine 200 mg once a day, and calcium lactate once a day. The examinations in 2020 showed moderate restriction from the pulmonary function test, bilateral lung parenchymal infiltrates from the chest x-ray, and ground-glass opacity from the chest computed tomography scan (CT-scan). The patient was monthly prescribed cyclophosphamide 500 mg intravenously for six months as the substitution of azathioprine, methylprednisolone 4 mg once a day, and aspirin 100 mg once a day. The latest worsening condition led the patient to receive the substitution of cyclophosphamide which was mycophenolate mofetil 360 mg twice a day, along with aspirin 100mg and methylprednisolone 4 mg once a day, respectively.

Conclusion: As a rare disease, the complications of systemic sclerosis should be anticipated. In this case, interstitial lung disease occurred following nearly five years of systemic sclerosis course which required immunosuppressive agents to enhance the efficacy of treatment. Comprehensive treatment with tight monitoring is necessary to slow the disease progression.


  1. Rosendahl AH, Schönborn K, Krieg T. Pathophysiology of systemic sclerosis (scleroderma). Kaohsiung J Med Sci. 2022;38(3):187-195.
  2. Frans JV. Etiology and Pathogenesis of Scleroderma. In: Firestein G BR, Gabriel S, McInnes I, O’Dell J, ed. Kelley & Firestein’s Textbook of Rheumatology 10th Edition. Philadelphia: Elsevier; 2017: 1400–23.
  3. Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc-ILD). Respiratory research. 2019;20:1-10.
  4. Peoples C, Medsger Jr TA, Lucas M, Rosario BL, Feghali-Bostwick CA. Gender differences in systemic sclerosis: relationship to clinical features, serologic status and outcomes. Journal of scleroderma and related disorders. 2016;1(2):204-12.
  5. Johnson SR. New ACR EULAR guidelines for systemic sclerosis classification. Current rheumatology reports. 2015;17:1-8.
  6. Anggoro A, Hasan H. Interstitial lung disease in Indonesian adult with systemic sclerotic: A rare case. Annals of Medicine and Surgery. 2022;75:103386.
  7. Allanore Y, Simms R, Distler O, et al. Systemic sclerosis. Nature reviews Disease primers. 2015;1(1):1-21.
  8. Hax V, Bredemeier M, Moro ALD, et al. Clinical algorithms for diagnosing and prognosis interstitial lung disease in systemic sclerosis. Seminars in Arthritis and Rheumatism; 2017: Elsevier; 2017. p. 228-34.
  9. Solomon AW, Peeling RW, Foster A, Mabey DCW. Diagnosis and Assessment of Trachoma. Clinical Microbiology Reviews. 2004;17(4):982-1011.
  10. Wigley FM, Boin F. Clinical features and treatment of scleroderma. Kelley and Firestein's Textbook of Rheumatology: Elsevier; 2017:1424-60.
  11. Gutsche M, Rosen GD, Swigris JJ. Connective tissue disease-associated interstitial lung disease: a review. Current respiratory care reports. 2012;1:224-32.
  12. Hidayat R, Isbagio H, Ariane A, et al. Characteristics of patients with autoimmune rheumatic disease in the era of COVID-19 pandemic in Indonesia. Indonesian Journal of Rheumatology. 2020;12(1):1-9.
  13. Ginting AR, Tandiono V. Polymyositis concomitant with hepatitis B virus infection: Treatment challenges. Narra J, 2023;3(3):e514-e.
  14. Kamadjaja MJK, Pertiwi FN. Prosthodontic Management of a Completely Edentulous Patient with Microstomia and Flat Ridge Mandible: A Case Report. Acta Medica Philippina. 2019;53(6):1-8.
  15. Suliman YA, Dobrota R, Huscher D, et al. Pulmonary function tests: high rate of false negatives in the early detection and screening of scleroderma interstitial lung disease. Swiss Medical Weekly; 2015: Emh Swiss Medical Publishers Ltd Farnsburgerstr 8, Ch-4132 Muttenz, Switzerland; 2015. P. 9s-S.
  16. Naidu G, Sharma SK, Adarsh M, et al. Effect of mycophenolate mofetil (MMF) on systemic sclerosis-related interstitial lung disease with mildly impaired lung function: a double-blind, placebo-controlled, randomized trial. Rheumatology International. 2020; 40(2):207-16.
  17. Distler O, Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis–associated interstitial lung disease. New England Journal of Medicine. 2019;380(26): 2518-28.
  18. Bernstein EJ, Peterson ER, Sell JL, et al. Survival of adults with systemic sclerosis following lung transplantation: a nationwide cohort study. Arthritis & rheumatology. 2015; 67(5):1314-22.

How to Cite

Retnaningtyas, F. D., & Awalia. (2024). Diagnostic approaches and treatments of systemic sclerosis patient with interstitial lung disease. Bali Medical Journal, 13(1), 877–881.




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