Hemosiderosis overview

Hemosiderosis Microchapters
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Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Hemosiderosis from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Physical Examination
Laboratory Findings
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Medical Therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

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. 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
• Group 2 pulmonary hemosiderosis
• Group 3 pulmonary hemosiderosis

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 majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

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].