Misleading Diagnosis of Idiopathic Pulmonary Fibrosis: A Clinical Concern
Diagnosis of Sjogren syndrome should be based on consideration of the clinical presentation, a pulmonary function test, blood and rheumatology laboratory findings, radiographic imaging patterns, and biopsy results.
Dr. Ramos-Rossy, Dr. Otero-Dominguez, and Dr. Baez-Corujo are Pulmonary and Critical Care Medicine Fellow Physicians; Dr. Cantres-Fonseca and Dr. Rodríguez- Cintrón are Pulmonary and Critical Care Medicine Attending Physicians; and Ms. Arzon-Nieves is a Research Study Coordinator, all at the VA Caribbean Healthcare System in San Juan, Puerto Rico. Correspondence: Dr. Ramos-Rossy (Javier.Ramos-Rossy @va.gov)
Author disclosures The authors report no actual or potential conflicts of interest with regard to this article.
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Diagnosing IPF cannot be solely based on a lung biopsy consistent with UIP. Appropriate diagnosis should consider the clinical presentation; PFT, laboratory findings (including rheumatologic workup), imaging (especially radiographic patterns), and biopsies. Moreover, the pathologic characteristic of IPF, which is UIP, can be found with other diseases, such as SS. Thus, it is important to make an accurate diagnosis to provide the appropriate treatment available. Patients with ILD associated with SS who have worsening symptoms, PFT, and radiographic abnormalities may be treated with oral prednisone (daily dose: 1 mg/kg).
Conclusion
This case highlights the importance of making an adequate diagnosis of ILD considering that available treatments differ for all possible etiologies other than IPF. This is a true clinical concern taking into account that many patients might be receiving inappropriate therapy for IPF diagnosis, as illustrated in the case study.
