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Colegio de Medicina Interna de México.

2024, Number 3

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Med Int Mex 2024; 40 (3)

Abatacept in shrinking lung syndrome

Pimentel LRR, García CM, Chávez SIN

Language: Spanish
References: 13
Page: 232-237
PDF size: 573.37 Kb.


ABSTRACT

Background: Shrinking lung syndrome is a condition that occurs in patients with systemic lupus erythematosus, it is the least frequent pulmonary manifestation reported in about 1%. It is characterized by dyspnea and pleuritic chest pain; the characteristic radiographic change is the elevation of the hemidiaphragms, the tomography shows decreased lung volumes and a restrictive pattern is observed in the spirometry; the diagnosis requires a high index of suspicion and a careful evaluation. There is no standardized treatment, but steroids and immunosuppressants such as azathioprine, cyclosporine, methotrexate and cyclophosphamide have been prescribed, in addition to biological therapies such as rituximab. Patients generally have a good response, but refractory cases have been described.
Clinical case: A 33-year-old female patient with a diagnosis of systemic lupus erythematosus and symptoms characterized by effort dyspnea, chest pain, absence of air entry in the lung bases, radiography with elevation of the hemidiaphragms and a restrictive pattern in spirometry. Shrinking lung syndrome was concluded, the management was with steroids and immunosuppressants without improvement and patient had an adverse effect with rituximab and a good response to the administration of abatacept was observed.
Conclusions: Shrinking lung syndrome must be taken into consideration in the approach to patients with systemic lupus erythematosus and dyspnea, Abatacept could be a therapeutic option in refractory cases.


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