Interstitial Pneumonia with Autoimmune Features (IPAF) and Radiological Findings Suggestive of Lymphocytic Interstitial Pneumonia (LIP)—Case Report

Abstract

Interstitial pneumonia with autoimmune features (IPAF) is a term to describe individuals with both interstitial lung disease (ILD) and combinations of other clinical, serologic, and/or pulmonary morphologic features, which presumably originate from an underlying systemic autoimmune condition, but do not meet current rheumatologic criteria for a defined connective tissue disease (CTD). Predominantly, interstitial pneumonia arises in the course of an established CTD, but it is not so rare for the ILD to be the first, and possibly the one and only manifestation of a latent CTD. Lymphocytic Interstitial Pneumonia (LIP) is an uncommon disease, characterized by infiltration of the interstitium and alveolar spaces of the lung by lymphocytes, plasma cells and other lymphoreticular elements. The cause of LIP is still unknown but it could be also a manifestation of CTD. Clinically, it is highly variable, from spontaneous resolution to progressive respiratory failure and death despite glucocorticoid treatment. Since there are no recent standards for the management of LIP, the disease is treated empirically. We report a case of a HIV-negative 54-year-old woman, who was suspected of LIP according to clinical features and radiological findings. Positive laboratory results were highly suggestive of underlying autoimmune process, but did not fulfil the criteria of any particular CTD. Because of severe general condition of the patient, immunosuppressive treatment was started immediately, without further invasive diagnostics including lung biopsy, which is required for a definitive diagnosis. We present two-year observation of the patient with all our doubts concerning clinical proceedings.

A 63-Year-Old Woman With an Acute Exacerbation of Interstitial Pneumonia

“…Although we occasionally encounter patients with autoimmune disease preceded by pulmonary involvement, it is very difficult to speculate on the underlying autoimmune diseases at the onset of lung involvements. Autoimmune disease-associated LIP preceding lung involvement is extremely rare, to our knowledge, with only two cases of primary biliary cirrhosis-associated LIP described by Koss et al 5 Płóciniczak et al 6 described a case of LIP that was diagnosed as interstitial pneumonia with autoimmune features, and such a case may meet the diagnostic criteria for specific autoimmune disease after long-term follow-up. Patients who are diagnosed with idiopathic LIP should be followed up carefully, as idiopathic LIP may later develop into autoimmune disease.…”

Biomarcadores patológicos da pneumonite intersticial bronquiolocêntrica

“…Embora uma doença intersticial costume surgir no decurso da doença autoimune, não é raro que a DPI a anteceda em anos (28,29). Por isso, a ERS/ATS em 2015 (30,31)…”