Idiopathic pulmonary hemosiderosis: challenges of differential diagnosis
A. A. Kuznetsova,
S. I. Petrova,
M. F. Dubko,
I. I. Grafskaya,
N. M. Solomatina,
A. A. Bukovskaya,
O. F. Pozdnyakova,
O. L. Krasnogorskaya,
E. P. Fedotova,
Yu. B. Klyukhina,
M. V. Kuropatenko,
Yu. V. Peshekhonova,
L. A. Zhelenina,
M. M. Kostik https://doi.org/10.18093/0869-0189-2024-4444
Abstract
Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unclear etiology that belongs to interstitial lung diseases and is characterized by a triad of symptoms: hemoptysis, iron deficiency anemia, and diffuse interstitial changes on computed tomography (CT) of the chest cavity. IPH can occur at any age, but the disease develops in childhood in 80% of cases, more often before 10 years of age. A significant proportion of pulmonary hemosiderosis cases in this age group remain undiagnosed. The purpose of the study is to demonstrate a clinical case illustrating the features of differential diagnosis and therapy of IPH during the COVID-19 pandemic. Conclusion. An 8-year-old girl was diagnosed with IPH based on fever, shortness of breath, weakness, and severe iron-deficient anemia. The chest CT scan revealed diffuse interstitial ground-glass opacity in lungs. The lung biopsy revealed hemosiderin deposits and hemosiderophages in the alveolar lumens. Treatment with systemic glucocorticoids (SGC) and azathioprine was effective. The patient was followed for 3 years.
Keywords
Supporting agencies: The authors declare that they did not receive any external funding for the study and preparation of the publication.
About the Authors
A. A. Kuznetsova
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Alla A. Kuznetsova, Doctor of Medicine, Professor of the Department of Faculty Pediatrics
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (812) 416-52-72
Competing Interests:
No conflict of interest is declared by the authors.
S. I. Petrova
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Sveylana I. Petrova, Candidate of Medicine, Associate Professor, Department of Faculty Pediatrics
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (812) 416-52-72
Competing Interests:
No conflict of interest is declared by the authors.
M. F. Dubko
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Margarita F. Dubko, Candidate of Medicine, Associate Professor of the Department of Hospital Pediatrics
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (812) 416-52-12
Competing Interests:
No conflict of interest is declared by the authors.
I. I. Grafskaya
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Irina I. Grafskaya, Pediatrician, Pulmonologist, Pediatric Department No. 2
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (812) 416-52-72
Competing Interests:
No conflict of interest is declared by the authors.
N. M. Solomatina
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Natalya M. Solomatina, Pediatrician, Pulmonologist, Pediatric Department No. 2
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (812) 416-52-72
Competing Interests:
No conflict of interest is declared by the authors.
A. A. Bukovskaya
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Anastasiya A. Bukovskaya, Clinical Resident, Department of Faculty Pediatrics
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (911) 766-88-28
Competing Interests:
No conflict of interest is declared by the authors.
O. F. Pozdnyakova
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Olga F. Pozdnyakova, Candidate of Medicine, Associate Professor, Department of Medical Biophysics and Physics, Radiologist, Department of Functional and Radiation Diagnostics with Computer Tomography Rooms
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (812) 416-53-92
Competing Interests:
No conflict of interest is declared by the authors.
O. L. Krasnogorskaya
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Olga L. Krasnogorskaya, Candidate of Medicine, Associate Professor, Department of Pathological Anatomy with a Course in Forensic Medicine named after Professor D.D.Lokhov, Head of the Pathological Anatomy Department of the Clinic
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (812) 416-52-74
Competing Interests:
No conflict of interest is declared by the authors.
E. P. Fedotova
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Elena P. Fedotova, Associate Professor, Department of Pathological Anatomy with a Course of Forensic Medicine named after Professor D.D.Lokhov
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (812) 416-52-74
Competing Interests:
No conflict of interest is declared by the authors.
Yu. B. Klyukhina
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Yulia B. Klyukhina, Candidate of Medicine, Chief Freelance Pediatric Pulmonologist of the Health Committee of Saint Petersburg, Assistant, Department of Children’s Diseases named after Professor I.M.Vorontsov, Faculty of Postgraduate and Further Professional Education
Author ID: 908122;
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (812) 295-14-04
Competing Interests:
No conflict of interest is declared by the authors.
M. V. Kuropatenko
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation; Federal State Budgetary Scientific Institution “Institute of Experimental Medicine”, Ministry of Science and Higher Education of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Mariya V. Kuropatenko, Candidate of Medicine, Associate Professor, Fellow Worker of the Department of Children’s Diseases named after Professor I.M.Vorontsov, Faculty of Postgraduate and Further Professional Education; Leading Researcher, Laboratory of Biofeedback Physiology, Department of Ecological Physiology
Scopus ID: 57222538102;
ul. Litovskaya 2, Saint-Petersburg, 194100;
ul. Akademika Pavlova 12, Saint-Petersburg, 197022;
tel.: (812) 234-09-25
Competing Interests:
No conflict of interest is declared by the authors.
Yu. V. Peshekhonova
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Yulia V. Peshekhonova, Candidate of Medicine, Head of the Pediatric Department No. 2, Clinic
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (812) 416-52-72
Competing Interests:
No conflict of interest is declared by the authors.
L. A. Zhelenina
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Liudmila A. Zhelenina, Doctor of Medicine, Professor, Department of Children’s Diseases named after Professor I.M.Vorontsov, Faculty of Postgraduate and Further Professional Education
Author ID: 108976;
Scopus ID: 6505996764;
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (812) 295-14-04
Competing Interests:
No conflict of interest is declared by the authors.
M. M. Kostik
Federal State Budgetary Educational Institution of Higher Education “Saint Petersburg State Pediatric Medical University” Ministry of Heath of the Russian Federation
Russian Federation
Competing Interests:
Russian Federation
Mikhail M. Kostik, Doctor of Medicine, Professor, Professor of the Department of Hospital Pediatrics
ul. Litovskaya 2, Saint-Petersburg, 194100;
tel.: (812) 416-52-12
Competing Interests:
No conflict of interest is declared by the authors.
References
1. Kuznetsova A.A., Pakhomova A.A., Petrova S.I., Peshekhonova Yu.V. [Neuroendocrine cell hyperplasia of infancy]. Pediatr. 2016; 7 (2): 170–175. DOI: 10.17816/PED72170-175 (in Russian).
2. Sadykova D.I., Makarova T.P., Shakirova A.R. et al. [Idiopathic hemosiderosis of the lung tissue]. Rossiyskiy vestnik perinatologii i pediatrii. 2021; 66 (5): 219–222. DOI: 10.21508/1027-4065-2021-66-5-219-222 (in Russian).
3. Malyuzhinskaya N.V., Morgunova M.A., Petrova I.V., Klitochenko G.V. [Idiopathic pulmonary hemosiderosis in childhood (clinical cases)]. Onkogematologiya. 2022; 17 (1): 82–86. DOI: 10.17650/1818-8346-2022-17-1-82-86 (in Russian).
4. Deutsch G.H., Young L.R., Deterding R.R. et al. Diffuse lung disease in young children: application of a novel classification scheme. Am. J. Respir. Crit. Care Med. 2007; 176 (11): 1120–1128. DOI: 10.1164/rccm.200703-393oc.
5. Saha B. K. Idiopathic pulmonary hemosiderosis: a state of the art review. Respir. Med. 2021; 176: 106234. DOI: 10.1016/j.rmed.2020.106234.
6. Zhang Y., Luo F., Wang N. et al. Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients. J. Int. Med. Res. 2019; 47 (1): 293–302. DOI: 10.1177/0300060518800652.
7. Taytard J., Nathan N., de Blic J. et al. New insights into pediatric idiopathic pulmonary hemosiderosis: the French RespiRare(®) cohort. Orphanet J. Rare Dis. 2013; 8: 161–168. DOI: 10.1186/1750-1172-8-161.
8. Moissidis I., Chaidaroon D., Vichyanond P., Bahna S.L. Milk-induced pulmonary disease in infants (Heiner syndrome). Pediatr. Allergy Immunol. 2005; 16 (6): 545–552. DOI: 10.1111/j.1399-3038.2005.00291.x.
9. Clement A., Nathan N., Epaud R. et al. Interstitial lung diseases in children. Orphanet. J. Rare Dis. 2010; 5: 22. DOI: 10.1186/1750-1172-5-22.
10. Eldem I., Ileri T., Ince E. et al. Idiopathic pulmonary hemosiderosis with allergic asthma diagnosis in a pediatric patient. J. Pediatr. Hematol. Oncol. 2015; 37 (7): e435–437. DOI: 10.1097/MPH.0000000000000412.
11. Tryfon S., Papadopoulou E., Psarros G. et al. Celiac disease and idiopathic pulmonary hemosiderosis: a literature review of the Lane–Hamilton syndrome. Postgrad. Med. 2022; 134 (8): 732–742. DOI: 10.1080/00325481.2022.210912.
12. Bakalli I., Kota L., Sala D. et al. Idiopathic pulmonary hemosiderosis – a diagnostic challenge. Ital. J. Pediatr. 2014; 40: 35–39. DOI: 10.1186/1824-7288-40-35.
13. Siu K.K., Li R., Lam S.Y. Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis. Hong Kong Med. J. 2015; 21 (2): 172–174. DOI: 10.12809/hkmj144237.
14. Kania A., Misiaszek M., Vašáková M. et al. Cryobiopsy in the diagnosis of idiopathic pulmonary hemosiderosis: a case report. J. Thorac. Dis. 2019; 11 (7): 3195–3201. DOI: 10.21037/jtd.2019.07.17.
15. Sharp C., McCabe M., Adamali H., Medford A.R. Use of transbronchial cryobiopsy in the diagnosis of interstitial lung disease – a systematic review and cost analysis. QJM. 2017; 110 (4): 207–214. DOI: 10.1093/qjmed/hcw142.
16. Hetzel J., Maldonado F., Ravaglia C. et al. Transbronchial cryobiopsies for the diagnosis of diffuse parenchymal lung diseases: Expert statement from the cryobiopsy working group on safety and utility and a call for standardization of the procedure. RES. 2018; 95 (3): 188–200. DOI: 10.1159/000484055.
17. Zhang Y., Luo F., Wang N. et al. Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients. J. Int. Med. Res. 2019; 47 (1): 293–302. DOI: 10.1177/0300060518800652.
18. AlJassmi A.M. Autoimmunity and delayed diagnosis in pediatric idiopathic pulmonary hemosiderosis. J. Pediatr. Hematol. Oncol. 2020; 42 (4): e240–243. DOI: 10.1097/MPH.0000000000001513.
19. Noronha N., Ramalho P., Barreira R. et al. Severe iron-deficiency anemia as initial manifestation of pulmonary hemosiderosis in a child. Einstein (Sao Paulo). 2018; 16 (4): eRC4505. DOI: 10.31744/einstein_journal/2018RC4505.
20. Castellazzi L., Patria M.F., Frati G. et al. Idiopathic pulmonary haemosiderosis in paediatric patients: how to make an early diagnosis. Ital. J. Pediatr. 2016; 42 (1): 86. DOI: 10.1186/s13052-016-0296-x.
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Kuznetsova A.A., Petrova S.I., Dubko M.F., Grafskaya I.I., Solomatina N.M., Bukovskaya A.A., Pozdnyakova O.F., Krasnogorskaya O.L., Fedotova E.P., Klyukhina Yu.B., Kuropatenko M.V., Peshekhonova Yu.V., Zhelenina L.A., Kostik M.M. Idiopathic pulmonary hemosiderosis: challenges of differential diagnosis. PULMONOLOGIYA. 2025. (In Russ.) https://doi.org/10.18093/0869-0189-2024-4444
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