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. Hemosiderosis occurs in the setting of genetic disorders (ie hemochromatosis occurs due to excessive iron absorption), transfusional, abnormal clearance/use, increase absorption, abnormal hepcidin, hemolytic anemia, or homeotropic parasites. Although hemosiderosis implies iron overload without tissue damage, often an early stage of iron accumulation, there are three types of hemosiderosis: Transfusion hemosiderosis, Idiopathic pulmonary hemosiderosis, Transfusional diabetes. Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology characterized by repeated episodes of a diffuse alveolar hemorrhage which cause periodic attack of tachycardia, pyrexia, pallor, fatigue, cyanosis, increasing dyspnea, signs of congestive cardiac failure, severe anamia and hemoptysis. Repeated alveolar hemorrhage causes the accumulation of hemosiderin, a by-product of hemoglobin breakdown, in the alveoli.Alveolar macrophages take up these hemosiderin molecules, usually within 36 - 72 hours, and can remain in the lungs for up to 8 weeks. Between attacks patient may remain well but commonly there is chronic ill-health. Over the time, It can lead to multiple respiratory complications and permanent lung damage. It is not familial and is found primarily in children from a few months to 16 years of age and rarely be seen in adults.[1] [2]
Historical Perspective
- IPH was first described as "brown lung induration" by Rudolf Virchow in 1864 in patients after their death.[3]
- The findings were first to the clinical symptoms by Wilhelm Ceelen gave a more detailed description of the condition after autopsies revealed large amounts of hemosiderin in 2 children in 1931.
- In 1944, the antemortem diagnosis was made by Waldenstrom
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
- The clinical spectrum of IPH ranges from asymptomatic cases to a chronic cough and dyspnea to repetitive hemoptysis with fatigue, anemia, and slowly progressive dyspnea and life-threatening acute respiratory failure.
- 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 acute IPH is made clinically, after other known causes of pulmonary and systemic or visceral hemorrhage have been excluded.
Symptoms
The clinical features of idiopathic pulmonary hemorrhage depend on whether it is acute or chronic Symptoms of IPH in the acute phase may include the following:
- Severe dyspnea
- Cough
- Hemoptysis
- Worsening anemia
- Rapid asphyxiation due to massive pulmonary hemorrhage
patients who present in the chronic phase of the disease may have:
- Weight loss
- Failure to thrive
- Hypoxemic respiratory failure in severe cases,
Physical Examination
Physical examination findings vary depending on whether it is an acute or chronic presentation.
- In the acute phase, the physical examination may be completely normal or include respiratory signs such as tachypnea.
- In the chronic phase, there may be:
- Pallor
- Failure to thrive
- Weight loss
- Hepatosplenomegaly
- Digital clubbing and other signs of chronic hypoxia in the setting of pulmonary fibrosis
Laboratory Findings
- Reduced hemoglobin counts and hematocrit, leucocytosis, and elevated erythrocyte sedimentation rate may be seen in patients with IPH.
- Erythrocytes and hemosiderin-laden macrophages may be seen in sputum testing with hematoxylin-eosin and Prussian blue stains, indicating intra-alveolar bleeding. Stains and cultures for mycobacteria, bacteria, and fungus are done to rule out the infective cause.
- Alveolar macrophages filled with hemosiderin and intact erythrocytes and occasionally neutrophils in Bronchoalveolar lavage (BAL) has a higher diagnostic yield than sputum testing.
- Pulmonary function testing generally shows a restrictive pattern of varying severity. The carbon monoxide diffusing capacity (DLCO) can be elevated during the acute phase.
Imaging Findings
- Chest x-ray taken during an acute phase of IPH exacerbation may show diffuse alveolar infiltrates greatest at the lung bases.
- CT scans taken during an acute phase of IPH exacerbation may show ground-glass attenuation in the lung bases.
- Chromium and technetium based perfusion scans show intra-alveolar bleeding. Scans with abnormal pulmonary uptake 12-24 hours after the injection have been observed in patients with pulmonary hemorrhage.
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.
- ↑ McLETCHIE NG, COLPITTS G (August 1949). "Essential brown induration of the lungs; idiopathic pulmonary haemosiderosis". Can Med Assoc J. 61 (2): 129–33. PMC 1591635. PMID 18147236.
- ↑ Madu A, Siddiqui AH. PMID 32491447 Check
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