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Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive and inevitably fatal lung disease. Although the etiology and pathogenesis of IPF remain incompletely understood, two drugs (e.g., pirfenidone and nintedanib) have proven effective in slowing down functional decline and disease progression and are now approved worldwide for treatment. Yet, as outlined by the recent guideline document on treatment of IPF, every therapeutic decision needs to be tailored to the individual patient, after discussing potential benefits and pitfalls. Comorbidities, which almost invariably complicate IPF, impact significantly clinical course and prognosis of the disease, making a holistic approach to the best care for these patients. Randomized-controlled trials remain a valid choice for selected IPF patients and their completion is critically important to achieving the ultimate goal of improving both survival and quality of life of patients suffering from this devastating disease.

Idiopathic pulmonary fibrosis (IPF) is a deadly progressive lung disease without an effective standard treatment approach. Because of the complexity and uncertainties of IPF treatment, therapeutic decisions need to be tailored to the individual patient, after discussing the potential benefits and pitfalls. Pirfenidone has been approved for the treatment of IPF in many countries, but is not recommended as a first-choice therapy by current guidelines because of the lack of a definite efficacy. Randomized controlled trials represent a valid choice for patients with IPF, and their completion is important in improving both survival and quality of life.

Bronchoalveolar lavage (BAL) has only a limited role in diagnosis of idiopathic pulmonary fibrosis (IPF). A finding of raised neutrophils (>5%) and eosinophils (>2%) is characteristic but not diagnostic of IPF. BAL cell count does not clearly differentiate between fibrotic non-specific interstitial pneumonia and IPF either diagnostically or prognostically. BAL in IPF should be considered in all patients with suspected infection, malignancy or acute exacerbations. In such cases, it may be diagnostic. Because of few and conflicting results BAL fluid analysis has very little clinical relevance determining prognosis and response to treatment in IPF