The Idiopathic Pneumonia Syndrome is a clinical syndrome that may be associated with a spectrum of histopathologic processes (diffuse alveolar damage with hyaline membranes, bronchiolitis obliterans, interstitial pneumonitis, diffuse alveolar hemorrhage, delayed pulmonary toxicity syndrome, noncardiogenic capillary leak syndrome).
Patient selection: bone marrow or stem cell transplantation
Primary feature is evidence of diffuse lung injury:
(1) multilobar infiltrates on imaging studies
(2) signs and symptoms of pneumonia, including cough, rales, and dyspnea
(3) abnormal pulmonary physiology - one or both of the following:
(3a) increased alveolar to arterial oxygen gradient
(3b) new or increased restrictive lung findings
No evidence of the following (exclusions):
(1) lower respiratory tract infection
(1a) failure to improve after therapy with broad-spectrum antibiotics
(1b) cultures from broncho-alveolar lavage are negative for bacterial, viral and fungal pathogens
(1c) lung biopsy negative for infectious agents
(1d) ideally a second evaluation conducted 2 to 14 days after the initial evaluation is negative for infectious agents
(2) cardiogenic cause
