A Case of Interstitial Pneumonia with Features of Autoimmunity

We present a case of a 61-year-old woman with several months of gradually worsening shortness of breath, requiring multiple hospitalizations with acute hypoxemic respiratory failure. She was initially treated for eosinophilic pneumonia presumed to be secondary to medications or rheumatoid lung without much improvement. Her subsequent chest CT showed honeycombing and diffuse ground-glass opacities, and she was found to have elevated rheumatoid factor (RF) and anti-CCP antibody titers without extrathoracic features of rheumatoid arthritis. This clinical scenario was suggestive of an interstitial lung disease (ILD) due to occult underlying connective tissue disorder (CTD), along the lines of the recently proposed entity interstitial pneumonia with autoimmune features (IPAF). She continued to deteriorate rapidly and passed away after experiencing recurrent exacerbations. As there is limited evidence to explain the clinical course of such patients, there is a need for prospective research to develop tailored regimens to prevent progression or even reverse the disease process.