Introduction
Some patients with an idiopathic interstitial pneumonia (IIP) have
autoimmune features but do not fulfill established criteria for a
connective tissue disease (CTD) (1). In 2015, the « European Respiratory
Society (ERS) and American Thoracic Society (ATS) Task Force on
Undifferentiated Forms of Connective tissue disease-associated
interstitial lung disease » proposed the term « interstitial pneumonia
with autoimmune features » (IPAF) (2). The classification criteria for
IPAF is a combination of three domains : clinical features, serologic
features and morphologic features. The physiopathology of the disease
remains unclear and there is no specific treatment so that
corticosteroids and immunosuppressive agents are the mainstay of the
treatment (1).
Interstitial lung disease (ILD) is associated with considerable
morbidity and mortality. There can be a lack of therapeutic response in
very severe lung involvement despite maximal conventional treatment, in
such a way that rituximab (Rtx) may be an effective medical rescue
therapy for ILD unresponsive to conventional immunosupressive treatment
(3).
We present a patient with IPAF treated successfully with rituximab used
as salvage therapy.