*3.7. Differential Diagnosis*

In the differential diagnosis (DD) bronchial asthma, chronic aspiration, acute and chronic lower respiratory tract infections, including fungal ones, cystic fibrosis, foreign body, hypersensitivity pneumonitis, bronchopulmonary aspergillosis, secondary PH and IPH should be considered [11]. Cystic fibrosis essentially can be excluded by a normal chloride level on the sweat test and second line tests if there is a strong suspicion. Infectious causes should be ruled out with tuberculin skin test and available microbial tests, and empiric antibiotic treatments characteristically are inefficacious. Thereafter, a vasculitis or autoimmune disorder can be considered. The characteristic pulmonary hemorrhage attacks cannot be enlightened by modest bronchial asthma. The lack of chronic inhalant exposure and BAL examination can rule out hypersensitivity pneumonitis. Bronchopulmonary aspergillosis is unlikely if skin reactivity to *Aspergillus* antigens is negative or precipitating serum antibodies to *A. fumigatus* are absent. No hemorrhagic focus nor foreign bodies can be found on bronchoscopy. In particular, DD should consider IPH, that includes the classic triad hemoptysis, radiologic lung infiltrate and iron deficiency anemia, having a more severe course and prognosis [22]. Moreover, IPH occurs in older children, and it is rarely associated with gastrointestinal symptoms [23] (Table 2).


**Table 2.** Differential diagnosis between Heiner syndrome (HS) and idiopathic pulmonary hemosiderosis (IPH).
