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146KUWAIT MEDICAL JOURNAL June 2009Case ReportIdiopathic Pulmonary HemosiderosisAbdullah Almutairi 1 , Nasser Behbehani 1 , Tareq MMA Mohammed 21Department of Medicine, Mubarak Alkabeer Hospital, Kuwait2Department of Pathology, Mubarak Alkabeer Hospital, KuwaitABSTRACTIdiopathic pulmonary hemosiderosis (IPH) is a raredisorder that is usually characterized by the triad ofbilateral pulmonary infiltrates, hemoptysis and irondeficiency anemia. The disease is well known to affectthe pediatric age group with conflicting treatmentKuwait Medical Journal 2009; 41 (2): 146-148trials. We report a case of a 19 year- old girl withIPH and respiratory failure who had excellent initialresponse to systemic steroids. To the best of ourknowledge this is the first reported case in this agegroup in the region.KEY WORDS: anemia, hemoptysis, immunosuppressive agents, steroidINTRODUCTIONIdiopathic pulmonary hemosiderosis (IPH)is a rare disorder, which affects children in themajority of cases [1] . There are several reportedcases in the adult population. Typically, intensivesearch for aetiology ends up negative. Patientsusually present with cough and hemoptysis,but iron deficiency anemia could be the solepresentation [2,3] . This presentation in an adultmakes the diagnosis more difficult as other causesof alveolar hemorrhage need to be carefullyexcluded. We report the case of a 19 year-oldgirl who presented to Mubarak Hospital withhemoptysis and respiratory failure followed by areview of relevant literature.CASE REPORTMs. F is a 19-year-old girl, who was transferredto Mubarak hospital from another institutionwith respiratory failure. Her illness started fivedays prior to her presentation with fever, coughand shortness of breath. She described episodesof hemoptysis during her illness with few bloodclots occasionally. On further questioning, shedescribed similar attack three years ago, whereshe had been coughing streaks of blood and wasfebrile. This was followed by a protracted course ofmild hemoptysis for almost one year followed byspontaneous resolution. Since then she continued tofeel short of breath on exertion, but attributed it tothe iron deficiency anemia that she suffered from.Her anemia was severe enough to require bloodtransfusion twice. She denied any skin rash, jointpain or swelling, mouth or genital ulcers.On admission to the referring hospital, she wasfebrile, tired and short of breath. Her initial chestX-ray showed bilateral alveolar infiltrates (Fig. 1).She was initially managed as community acquiredpneumonia. Soon after admission, her conditiondeteriorated with marked hypoxemia requiring 70%inspired oxygen. CT chest revealed bilateral diffuseinfiltrates, mixed alveolar opacities and interstitialinfiltrates.She was then transferred to our facility forfurther workup and management. On arrival shewas significantly hypoxic, but hemodynamicallystable. On a FIO 2of 0.7 her O 2saturation was only91%. She was admitted to the intensive care unit andstarted on broad spectrum antibiotics (maxipimeand intravenous erythromycin). Bronchoscopywas performed and transbronchial biopsies weretaken. Her bronchoalveolar lavage done prior to thebiopsy was consistently bloody. Full immunologicalinvestigations were sent to exclude any pulmonaryvasculitis including: ANA, C and P ANCA, Anti-GBM, complements and rheumatoid factor. Thesewere all negative except for a non-specific weakpositive C ANCA and ANA. The transbronchialbiopsy showed extensive hemosiderin in the tissueas well as hemosiderin laden macrophages (Fig. 2).There was also marked interstitial fibrosis with noevidence of vasculitis or capillaritis.Address correspondence to:Dr Abdullah Almutairi, Department of Medicine, Mubarak Alkabeer Hospital, Al-Jabriyah, Kuwait. E-mail: doctorq8@gmail.com