Caplan Syndrome: Current Insights into Pathogenesis, Clinical Manifestations, Diagnosis, And Emerging Therapeutic Strategies

Author

Annarapu Sriramteja, Syeda Nishat Fathima


Abstract

 Caplan syndrome, also known as rheumatoid pneumoconiosis, is a rare occupational lung disease characterized by the coexistence of rheumatoid arthritis, pneumoconiosis, and multiple pulmonary nodules following prolonged exposure to coal dust, silica, or other mineral particulates. The syndrome exemplifies the interplay between environmental exposure and autoimmune mechanisms, involving macrophage activation, inflammasome signaling, protein citrullination, autoantibody production, and chronic inflammation. Clinically, patients present with cough, dyspnea, chest discomfort, or incidental radiographic findings, while diagnosis relies on occupational history, serological markers, characteristic imaging, and exclusion of infections, malignancy, and other pulmonary disorders. Management focuses on eliminating dust exposure, smoking cessation, disease-modifying antirheumatic therapy, pulmonary rehabilitation, and multidisciplinary care to preserve lung function and control rheumatoid disease. Emerging research on cytokine-targeted therapies, antifibrotic agents, and biomarker-guided diagnosis offers promising therapeutic opportunities. This review summarizes the epidemiology, pathogenesis, clinical features, diagnosis, differential diagnosis, current management strategies, and future research directions of Caplan syndrome.


Keywords

Caplan syndrome; Rheumatoid pneumoconiosis; Rheumatoid arthritis; Pneumoconiosis; Silica exposure; Coal dust; Occupational lung disease; Pulmonary nodules



Full Text:

Download Paper PDF


References


  1. CAPLAN A. (1953). Certain unusual radiological appearances in the chest of coal-miners suffering from rheumatoid arthritis. Thorax, 8(1), 29–37. https://doi.org/10.1136/thx.8.1.29
  2. Ronsmans, S., & Blanc, P. D. (2023). Colinet-Caplan Syndrome: History of an Outbreak of Autoimmune Disease in Scouring Powder Workers. Annals of internal medicine, 176(2), 260–265. https://doi.org/10.7326/M22-2630
  3. Schreiber, J., Koschel, D., Kekow, J., Waldburg, N., Goette, A., & Merget, R. (2010). Rheumatoid pneumoconiosis (Caplan's syndrome). European journal of internal medicine, 21(3), 168–172. https://doi.org/10.1016/j.ejim.2010.02.004
  4. Laouali, A. M. M., Soumaila, M., Tinni, I. A., Clémence, N. N. T., Rosine, Z. L., Dembélé, O., Dimitri, S., Christèle, E. E., Rahanatou, M., Ouédraogo, I., Kadiatou, B., Giselle, B., & Martial, O. (2026). Caplan Syndrome: A Case Report. Clinical case reports, 14(1), e71864. https://doi.org/10.1002/ccr3.71864
  5. Deepak, J., & Kenaa, B. (2020). Caplan's Syndrome with a twist. International journal of clinical case reports and reviews : open access, 2(1), 10.31579/2690-4861/007. https://doi.org/10.31579/2690-4861/007
  6. De Capitani, E. M., Schweller, M., Silva, C. M., Metze, K., Cerqueira, E. M., & Bértolo, M. B. (2009). Rheumatoid pneumoconiosis (Caplan's syndrome) with a classical presentation. Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia, 35(9), 942–946. https://doi.org/10.1590/s1806-37132009000900017
  7. Baek, J., & Han, J. Y. (2024). Caplan Syndrome Mimicking Progressive Massive Fibrosis on CT: A Case Report. Journal of the Korean Society of Radiology, 85(4), 789–794. https://doi.org/10.3348/jksr.2023.0128
  8. Xiong, Z., Wu, Y., Tan, J., Chen, X., & Dong, J. (2026). Case Report: The entanglement of infection and autoimmunity: a case of Caplan syndrome. Frontiers in immunology, 17, 1715034. https://doi.org/10.3389/fimmu.2026.1715034
  9. Alfahad, A., Jennings, P., Smith, S., Niktash, N., & Curtin, J. (2015). An interesting finding of multiple calcified pulmonary nodules in a patient with rheumatoid arthritis. BJR case reports, 2(1), 20150116. https://doi.org/10.1259/bjrcr.20150116
  10. Williams, W.J. (1991), CAPLAN'S SYNDROME. International Journal of Clinical Practice, 45: 285-288. https://doi.org/10.1111/j.1742-1241.1991.tb08876.x
  11. Nemakayala, D. R., Surmachevska, N., Vaqar, S., & Ramphul, K. (2024). Caplan Syndrome. In StatPearls. StatPearls Publishing.
  12. Wang, L., Liu, Y., Zhang, Y., & Hu, Y. (2025). A four-year follow-up of Caplan's syndrome: A case report. The Journal of international medical research, 53(6), 3000605251353738. https://doi.org/10.1177/03000605251353738
  13. Min, Y. S., Kim, M. G., & Ahn, Y. S. (2021). Rheumatoid Arthritis in Silica-Exposed Workers. International journal of environmental research and public health, 18(23), 12776. https://doi.org/10.3390/ijerph182312776

 

 

 

Share your valuable work from Social Media Buttons