Our approach to the diagnosis of idiopathic pulmonary hemosiderosis (IPH)*

DAH: diffuse alveolar hemorrhage; CBC: complete blood count; BUN: blood urea nitrogen; Cr: creatinine; TIBC: total iron binding capacity; ANA: antinuclear antibody; ANCA: antineutrophil cytoplasmic antibodies; GBM: glomerular basement membrane; ds: double stranded; APLA: antiphospholipid antibodies; SLE: Systemic lupus erythematosus; APL: antiphospholipid; IgA: immunoglobulin A; TTG: tissue transglutaminase; TBLB: transbronchial lung biopsy; TBC: transbronchial cryobiopsy; VATS: video-assisted thoracoscopic surgery; BAL: bronchoalveolar lavage.
* Clinical manifestations of IPH:

• Hemoptysis (amount varies)
• Exertional dyspnea: may have recurrent episodes
• Iron deficiency anemia
• HRCT consistent with diffuse alveolar hemorrhage: patchy middle and lower lung zone opacities with air bronchogram; occasionally, subpleural honeycomb areas

¶ Iron deficiency anemia may be characterized by hypochromia, low serum iron. Sometimes serum ferritin is normal reflecting nonmobilizable hemosiderin in lungs. Refer to UpToDate discussion of IPH.
Δ Common causes of DAH: ANCA associated vasculitis, antiglomerular basement membrane (Goodpasture) disease, systemic lupus erythematosus, antiphospholipid antibody syndrome, cryoglobulinemia.
◊ The order of BAL and lab testing may vary; sometimes lab tests are requested, but results are not available at the time of BAL.
§ Refer to UpToDate content on diffuse alveolar hemorrhage.
¥ Patients with IgA deficiency can have a false negative test for IgA anti-TTG antibodies. Refer to UpToDate content for other ways to make the diagnosis of celiac disease.
‡ Refer to UpToDate content on the evaluation of diffuse lung disease in children and adults.