Bronchoalveolar lavage (BAL) cell counts, once thought to have prognostic value, do not appear to be a reliable indicator of prognosis [ 16,18-20 ]. Although one retrospective study reported that increased eosinophils in the BAL fluid was associated with a poorer prognosis, a larger prospective cohort study of 141 patients with SSc-ILD found that the proportion of eosinophils in the BAL fluid did not correlate with mortality, rate of functional deterioration, or progression-free survival [ 16,18 ]. An increased proportion of neutrophils in BAL fluid was associated with more extensive lung disease on HRCT, a greater reduction in DLCO, and early mortality (HR , 95% CI -), but it did not predict the rate of functional deterioration or progression-free survival [ 18 ]. In a prospective clinical trial of 158 patients with early-stage SSc and symptomatic lung involvement, the presence or absence of BAL fluid neutrophilia did not predict rate of worsening or response to therapy [ 21 ].
The treatment and prognosis of NSIP will be reviewed here. The clinical manifestations, evaluation, and diagnosis of NSIP and the diagnosis and management of the other IIPs are discussed separately. (See "Causes, clinical manifestations, evaluation, and diagnosis of nonspecific interstitial pneumonia" and "Idiopathic interstitial pneumonias: Clinical manifestations and pathology" and "Clinical manifestations and diagnosis of idiopathic pulmonary fibrosis" and "Treatment of idiopathic pulmonary fibrosis" and "Respiratory bronchiolitis-associated interstitial lung disease" and "Cryptogenic organizing pneumonia" and "Acute interstitial pneumonia (Hamman-Rich syndrome)" .)