Table 2.
Literature Review
| Author, Year published | Sex | Ethnicity | Comorbidities in addition to T21 | Age at 1st DAH (years) | Presenting Symptoms | Known Antibody Testinga | Diagnosis | Treatment | Outcome |
|---|---|---|---|---|---|---|---|---|---|
| Alimi, 2018 [10] | 4 F, 5 M | French | 3 with PAH, 3 with cardiopathy | Mean: 2.92 +/− 3.45 | 2 with hemoptysis, all with dyspnea, 6 with hemoglobin < 7 | 6 with antibodies: ANCA, ANA, CCP, PR3, MPO, TTG-IgA, IgE-CMP | IPH |
All: glucocorticoids 1: hydroxychloroquine 3: mycophenolate mofetil, azathioprine, and/or cyclophosphamide |
3 without relapses 6 with relapses, of which 3 died |
| Zhang, 2019 [11] | – | Chinese | – | – | 1 of 107 children hospitalized with IPH at a single institution over a 21 year period | – | IPH | – | – |
| Hori, 2015 [12] | F | Japanese | PAH, history of Kawasaki disease | 2 | Pallor, anorexia, dyspnea, hemoptysis, nephritis | +MPO | AAV | azathioprine, glucocorticoids | Improved, stable at time of publication |
| Rayment, 2017 [13] | F | – | Repaired AVSD | 2.5 | Respiratory distress, anemia, thrombocytopenia | +ANCA | Isolated pulmonary capillaritis, bilateral pulmonary embolisms | glucocorticoids, rituximab, azathioprine | Improved, stable for at least 2.5 years |
| Watanabe, 2015 [14] | F | Japanese | Hypothyroidism, autism | 9 | Cough, emesis of bloody sputum, hypoxemia, fatigue | +ANA, dsDNA | IPH | glucocorticoids | Recurred with hemoptysis 2 weeks later, then stable at least 4 months |
| Galent, 1983 [15] | M | African American | PAH | 2 | Respiratory distress, blood-streaked sputum, fever, nasal discharge, cough | Elevated IgD antibody to milk proteins | Milk-related pulmonary hemosiderosis and cor pulmonale | Removal of cow’s milk from diet | Symptoms resolved. Re-introduction and removal repeated pattern. |
| Aceti, 2012 [16] | F | Italian | History of VSD, ASD, PDA, necrotizing enterocolitis | 4 | Recurrent anemia and respiratory distress, hemoptysis | Negative ANA, ANCA, anti-GBM, TTG-IgA | Idiopathic Pulmonary Hemosiderosis | glucocorticoids | Recurred within 7 months, added hydroxychloroquine, recurred within 1 year |
| Schwab, 1996 [17] | M | German | – | 6 | Recurrent pneumonia, hemoptysis, anemia, renal failure | +pANCA | AAV | glucocorticoids, cyclophosphamide | No recurrence in 2 years |
| Niimi, 1992 [18] | F | VSD | 17 | Dyspnea |
+ANA Negative RF, Anti-GBM |
glucocorticoids | 3 recurrences in 4 years | ||
| Sato, 1986 [19] | F | Japanese | – | 3 | Cough, wheeze, anemia | – | – | azathioprine, disodium cromoglycate inhalation, milk avoidance | 7 recurrences in 1 year |
| Koyama, 1995 [20] | F | Japanese | – | 9 | Fever, dyspnea, anemia, pallor | – | IPH | glucocorticoids | No recurrence in 20 months |
(−) = unknown
Abbreviations: PAH Pulmonary Arterial Hypertension, ANCA Anti-neutrophil cytoplasmic antibody, ANA Anti-nuclear antigen, CCP Anti-Cyclic Citrullinated Peptide Antibody, PR3 Serine Protease 3 antibody, MPO Anti-Myeloperoxidase Antibody, IgE-CMP Immunoglobulin E to Cow’s Milk Protein, IPH Idiopathic Pulmonary Hemosiderosis, AAV ANCA-Associated Vasculitis, AVSD Atrioventricular Septal Defect, dsDNA Anti-double stranded DNA antibody, IgD Immunoglobulin D, VSD Ventricular Septal Defect, ASD Atrial Septal Defect, PDA Patent Ductus Arteriosus, GBM Glomerular Basement Membrane, TTG-IgA Tissue Transglutaminase Immunoglobulin A Antibody, pANCA Perinuclear ANCA, RF Rheumatoid Factor
aif unlisted, test is unknown