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While cardiac disease is responsible for a majority of RA-related deaths, pulmonary disease is also a major contributor, accounting for approximately 10-20% of all mortality. Pulmonary complications occur in 60-80% of RA patients, many of whom are asymptomatic. 7-10 RA directly affects all anatomic compartments of the thorax, including the lung parenchyma, large and small airways, pleura, and less commonly the vasculature (Box 1). 11,12 In addition, pulmonary infection and drug-induced lung disease associated with immunosuppressive agents utilized for the treatment of RA can occur. RA-associated lung disease typically occurs within 5 years of RA diagnosis and may even precede joint disease in up to 20% of patients. 13-16 Respiratory symptoms may be masked by the patient's poor functional status from chronic joint and systemic inflammation, which may lead to delays in diagnosis. 17 It is therefore imperative for clinicians to regularly assess RA patients for signs and symptoms of pulmonary disease and, reciprocally, to consider CTD, including RA, when evaluating a patient with pulmonary disease of unknown etiology.

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BackgroundHyaluronic acid (HA), an extracellular matrix component, is degraded in response to local tissue injury or stress. In various animal models of lung injury, HA has been shown to play a mechanistic role in modulating inflammation and injury. While HA is present in the lungs of patients with acute respiratory distress syndrome (ARDS), its relationship to patient outcomes is unknown.MethodsWe studied 86 patients with ARDS previously enrolled in the Phase II Randomized Trial of Fish Oil in Patients with Acute Lung Injury (NCT00351533) at five North American medical centers. We examined paired serum and bronchoalveolar lavage fluid (BALF) samples obtained within 48 hours of diagnosis of ARDS. We evaluated the association of HA levels in serum and BALF with local (lung injury score (LIS)) and systemic (sequential organ failure assessment score (SOFA)) measures of organ dysfunction with regression analysis adjusting for age, sex, race, treatment group, and risk factor for ARDS.ResultsWe found that both day-0 circulating and alveolar levels of HA were associated with worsening LIS (p = 0.04 and p = 0.003, respectively), particularly via associations with degree of hypoxemia (p = 0.02 and p < 0.001, respectively) and set positive end-expiratory pressure (p = 0.01 and p = 0.02, respectively). Circulating HA was associated with SOFA score (p < 0.001), driven by associations with the respiratory (p = 0.02), coagulation (p < 0.001), liver (p = 0.006), and renal (p = 0.01) components. Notably, the alveolar HA levels were associated with the respiratory component of the SOFA score (p = 0.003) but not the composite SOFA score (p = 0.27).ConclusionsElevated alveolar levels of HA are associated with LIS while circulating levels are associated with both lung injury and SOFA scores. These findings suggest that HA has a potential role in both local and systemic organ dysfunction in patients with ARDS.Electronic supplementary materialThe online version of this article (doi:10.1186/s13054-017-1895-7) contains supplementary material, which is available to authorized users.

Idiopathic pulmonary fibrosis (IPF) is a pulmonary disease involving fibrotic changes of the lung with unknown etiology, for which there is no cure. The pathogenesis of IPF involves dysregulated wound healing, damage to lung epithelium, fibroblast/myofibroblast activation and excessive extracellular matrix production, leading to aberrant lung remodeling. The receptor-like protein tyrosine phosphatase eta (CD148) exerts antifibrotic effects in experimental pulmonary fibrosis via interactions with its ligand syndecan-2; however, the role of CD148 in fibroblast activation remains unknown.

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