Hemosiderosis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Hemosiderosis is a condition whenever there is an overload of iron which results in the deposition of hemosiderin in many organs and tissues.
Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology characterized by repeated episodes of a diffuse alveolar hemorrhage, which over time, can lead to multiple respiratory complications and permanent lung damage. It is found primarily in children and rarely be seen in adults.[1] Haemosiderosis implies iron overload without tissue damage, often an early stage of iron accumulation.
Historical Perspective
- [Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
- In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
- In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].
Classification
- IPH may be grouped into three categories based on disease characteristic:
- Group 1 pulmonary hemosiderosis involves PH with circulating anti-GMB antibodies.
- Group 2 pulmonary hemosiderosis involves PH with an immune complex disease such as systemic lupus erythematosus, SLE.
- Group 3 pulmonary hemosiderosis involves no demonstrable immune system involvement.
Pathophysiology
The pathogenesis of hemosiderosis is characterized by Iron deposition into tissues due to: Genetic (ie hemochromatosis), Transfusional, Abnormal clearance/use, Increase absorption, Abnormal Hepcidin, Hemolytic anemia, Hemotropic parasites.
Causes
- There are no established causes for IPH, but it is likely to be multifactorial
- Possible associations include toxic insecticides (based on epidemiological studies in rural Greece), premature birth, and fungal toxin( toxigenic fungus Stachybotrys atra) exposure.
Differentiating IPH from other Diseases
- IPH must be differentiated from other diseases that cause alveolar hemorrhage, such as those include infectious etiologies( ARDS, streptococcus pneumoniae, staphylococcus aureus, and legionella, influenza A and pneumocystis jirovecii), rheumatic disease such as systemic lupus erythematosus, antiphospholipid antibody syndrome, Goodpasture disease, microscopic, and granulomatous polyangiitis, and mixed cryoglobulinemias, drug-induced injury in medications such as medication such as amiodarone, nitrofurantoin, and infliximab. Penicillamine, thromboembolic disease, bleeding disorders, and neoplasms
Epidemiology and Demographics
- The prevalence and incidence of IPH are relatively unknown because of the rare nature.
Age
- IPH is more commonly observed among children. ( approximately 80% of cases are seen in children who are diagnosed in the first decade of life.)
- 205 of cases are adult-onset IPH.
Gender
- IPH affects males and females equally in childhood-onset IPH
- Adult-onset IPH are almost twice as many males as females.
- Males are more commonly affected with IPH than females in adult-onset IPH.
Race
- There is no racial predilection for IPH.
Risk Factors
- Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
Natural History, Complications and Prognosis
- Early clinical features vary from an acute onset illness with hemoptysis and dyspnea to a chronic cough and dyspnea to repetitive hemoptysis with fatigue, anemia, and slowly progressive dyspnea.
- Common complications of IPH include Iron deficiency anemia and pulmonary fibrosis.
- Prognosis is generally variable, and the mean survival rate of patients with IPH is 2.5 to 5 years after diagnosis. Deaths can occur from acute massive hemorrhage or after progressive pulmonary insufficiency and right heart failure.
Diagnosis
Diagnostic Criteria
- The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
- [criterion 1]
- [criterion 2]
- [criterion 3]
- [criterion 4]
Symptoms
- [Disease name] is usually asymptomatic.
- Symptoms of [disease name] may include the following:
- [symptom 1]
- [symptom 2]
- [symptom 3]
- [symptom 4]
- [symptom 5]
- [symptom 6]
Physical Examination
- Patients with [disease name] usually appear [general appearance].
- Physical examination may be remarkable for:
- [finding 1]
- [finding 2]
- [finding 3]
- [finding 4]
- [finding 5]
- [finding 6]
Laboratory Findings
- There are no specific laboratory findings associated with [disease name].
- A [positive/negative] [test name] is diagnostic of [disease name].
- An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
- Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
Imaging Findings
- There are no [imaging study] findings associated with [disease name].
- [Imaging study 1] is the imaging modality of choice for [disease name].
- On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
- [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
Other Diagnostic Studies
- [Disease name] may also be diagnosed using [diagnostic study name].
- Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
Treatment
Medical Therapy
- There is no treatment for [disease name]; the mainstay of therapy is supportive care.
- The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
- [Medical therapy 1] acts by [mechanism of action 1].
- Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
Surgery
- Surgery is the mainstay of therapy for [disease name].
- [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
- [Surgical procedure] can only be performed for patients with [disease stage] [disease name].
Prevention
- There are no primary preventive measures available for [disease name].
- Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
- Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
References
- ↑ Chen XY, Sun JM, Huang XJ (November 2017). "Idiopathic pulmonary hemosiderosis in adults: review of cases reported in the latest 15 years". Clin Respir J. 11 (6): 677–681. doi:10.1111/crj.12440. PMID 26692115.