Background: Sarcoidosis is usually a benign disease, but it is described that the lung is the most frequently affected organ and in some cases the disease can progress to pulmonary fibrosis. Systemic corticosteroids, immunosuppressants and/or monoclonal antibodies are used for its treatment. The mortality rate without treatment is 1% to 6%.
Objectives: To establish the frequency of appearance of the stages of radiological involvement in patients diagnosed with sarcoidosis at Ciudad Real University General Hospital (CRUGH) for 4 years, describe the treatment guidelines used and investigate the causes of death.
Methods: Retrospective descriptive study that includes patients treated at Pneumology, Rheumatology and Internal Medicine Services of CRUGH from January 2017 to January 2021. Sarcoidosis diagnosis has been established according to ATS/ERS/WASOG (1999) criteria: compatible clinical and radiological presentation, evidence of non-caseating granulomas and exclusion of other granulomatous disease. The variables have been collected in an anonymized database analyzed by SPSS program.
Results: We have included 47 patients, 24 men and 23 women, with 52.0 ± 14.3 years of age. All presented radiological pulmonary involvement and in 26 cases (55.3%) there were also extrapulmonary manifestations. The most frequent radiological pattern is stage II in 29 cases (67.7%, hilar adenopathies and pulmonary infiltrates); followed by stage I in 16 (34.1%, hilar adenopathies) and only 2 cases presented stage IV (4.2%, fibrosis).
Currently, 20 patients (42.5%) are without treatment: 9 (19.1%) have not required it due to hyliomediastinal lymph node involvement without associated symptoms, and 11 (23.4%) achieve remission with oral glucocorticoids [OCG] at doses recommended in clinical guidelines. The other 27 (57.5%) receive these regimens: 10 (21.3%) OCG at usual doses; 11 (23.4%) OCG and conventional immunosuppressants -metrotexate 9 (19.1%), azathioprine 1 (2.1%) and cyclophosphamide 1 (2.1%)-; 3 (6.4%) OCG and antimalarials (hydroxychloroquine); 2 (4.2%) monotherapy with azathioprine; and 1 (2.1%) biological treatment with infliximab.
Three patients died (6.4%): one aged 71 due to progression to pulmonary fibrosis (2.1%), another 2 due to pulmonary adenocarcinoma (47 years old) and due to adenocarcinoma of the rectum (81 years old).
Conclusion: The predominant radiological pattern in our cohort is stage II with hilar adenopathies and pulmonary infiltrates. The most used treatment is OCG, as monotherapy (44.7%) or combined (29.4%). The high frequency of cancer mortality stands out.
REFERENCES:
[1]Drent M, Crouser ED, Grunewald J. Challenges of Sarcoidosis and Its Management. N Engl J Med. 2021 Sep 9;385(11):1018-1032
[2]Crouser ED, Maier LA, Wilson KC et al. Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2020 Apr 15;201(8):e26-e51.
[3]Rahaghi FF, Baughman RP et al. Delphi consensus recommendations for a treatment algorithm in pulmonary sarcoidosis. Eur Respir Rev. 2020 Mar 20;29(155):190146.
[4]Hunninghake GW, Costabel U, Ando M 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 Sep;16(2):149-73.
Disclosure of Interests: Ana Isabel Rebollo Giménez: None declared, Lirios Sacristán-Bou Speakers bureau: LS has received honoraria for speaker bureaus from: Glaxosmithkline (GSK), Chiesi, Gebro Pharma, Booehringer Ingelheim, Roche, Rovi, Teva, David Bellido Pastrana Speakers bureau: DB has received honoraria for speaker bureau from Glaxosmithkline, Patricia Mata Calderón Speakers bureau: PM has received honoraria for speaker bureaus from: Novartis, GSK, Gebro, Boerhinger, Astrazeneca, Menarini, Ferrer, Isabel Domínguez Osorio: None declared, Isabel María Ordóñez Dios Speakers bureau: IO has received honoraria for speakers bureau from Novartis, GSK, boehringer ingelheim, Roche and Chiesi, Marta Orta Caamaño Speakers bureau: MO has received honoraria for speaker bureau from Glaxosmithkline and Chiesi
, Carlos Bujalance Cabrera Speakers bureau: CB has received honoraria for speaker bureau from Faes and GSK, Francisco Javier Lázaro Polo Speakers bureau: FL has received honoraria for speaker bureau from GSK, Astrazeneca and Sanofi