A 39‐year‐old white woman presented to the emergency department with complaints of weakness and fatigue. On physical examination, the patient had orthostatic changes, and lung examination revealed bibasilar crackles. Laboratory data were significant for HGB of 5.1 g/dL with low MCV. Three stool guaiac tests were negative. On further questioning, the patient stated she has been having hemoptysis for a few weeks. The patient was placed in isolation. Chest X‐ray showed bibasilar nodular infiltrates. The PPD was nonreactive, and 3 AFB smears were negative. Furthermore, EGD and colonoscopy were negative. The patient then underwent bronchoscopy, which demonstrated hemosiderin‐laden macrophages. An extensive workup for vasculitis and autoimmune diseases was negative. Echocardiogram was unremarkable. Patient was then diagnosed with idiopathic pulmonary hemosiderosis (IPH) and started on steroids, with clinical improvement and resolution of hemoptysis.
IPH is a an extremely rare syndrome, with an incidence of 0.24–1.23 per million in selected populations. Mean survival after diagnosis is 2.5‐5 years. IPH is characterized by a triad of hemoptysis, pulmonary infiltrates, and anemia. However, this triad is seen in fewer than half of cases. Etiology remains unclear, although autoimmune phenomenon has been postulated because of the increasing coexistence of other autoimmune diseases, especially celiac disease. Clinical presentation differs mainly in the acute versus the chronic phase of the syndrome. Acutely, patients may present with hemoptysis, dyspnea, cough, and in severe cases respiratory failure sometimes leading to death. The chronic phase, insidious in onset, is characterized by asymptomatic anemia, dyspnea on exertion, fatigue, pallor, crackles on examination, and clubbing. The anemia, which is a result of occult pulmonary hemorrhage, is often iron‐deficiency anemia with low MCV and low transferrin saturation levels, A chest X‐ray usually shows a reticulonodular pattern, whereas CT scintography reveals varying degrees of fibrosis and honeycombing. Bronchoscopy will show alveolar macrophages filled with hemosiderin. Because IPH is a diagnosis of exclusion, other causes of pulmonary hemorrhage such as Goodpasture syndrome, SLE, Wegener's granulomatosis, and hypersensitivity to cow's milk, should be excluded. Thus, a workup should include ANCA, RF, ANA, anti‐GBM, antigliadin, antireticulin, and cow's milk precipitins, ESR, and CRP. A cardiac echo to rule out mitral stenosis should also be part of the workup. The mainstay of treatment of IPH is steroids; however, some authors recommend the addition of azathio‐prine if symptoms persist.
The clinical course of IPH is variable. Although anemia may be the only presenting feature, respiratory failure, and death can occur. Because hospitalists are the first to evaluate such patients in the emergency department, maintaining a high degree of clinical suspicion and including the disease in the differential diagnosis are mandatory.
T. Hamieh, none; J. Do, none.
To cite this abstract:Hamieh T, Do N. An Unusual Cause of Iron‐Deficiency Anemia. Abstract published at Hospital Medicine 2009, May 14-17, Chicago, Ill. Abstract 159. Journal of Hospital Medicine. 2009; 4 (suppl 1). https://www.shmabstracts.com/abstract/an-unusual-cause-of-irondeficiency-anemia/. Accessed May 26, 2019.