Lymphangioleiomyomatosis: Difference between revisions
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{{SI}} | |||
{{Infobox medical condition | |||
| name = Lymphangioleiomyomatosis | |||
| image = [[File:Lymphangioleiomyomatosis.jpg|250px]] | |||
| caption = Micrograph of lymphangioleiomyomatosis | |||
| field = [[Pulmonology]] | |||
| synonyms = LAM | |||
| symptoms = [[Shortness of breath]], [[chest pain]], [[cough]], [[pneumothorax]], [[chylothorax]] | |||
| complications = [[Respiratory failure]], [[pulmonary hypertension]], [[kidney]] [[angiomyolipoma]] | |||
| onset = Typically in women of childbearing age | |||
| duration = Chronic | |||
| causes = [[Genetic mutation]] in the [[TSC1]] or [[TSC2]] genes | |||
| risks = Female gender, [[tuberous sclerosis complex]] | |||
| diagnosis = [[CT scan]], [[lung biopsy]], [[pulmonary function test]] | |||
| differential = [[Asthma]], [[chronic obstructive pulmonary disease]], [[interstitial lung disease]] | |||
| treatment = [[Sirolimus]], [[everolimus]], [[lung transplantation]] | |||
| medication = [[Bronchodilators]], [[hormonal therapy]] | |||
| prognosis = Variable, can lead to progressive [[lung disease]] | |||
| frequency = Rare, estimated 3-5 per million women | |||
| deaths = Can be fatal if untreated | |||
}} | |||
[[File:Lymphangioleiomyomatosis_-_low_mag.jpg|Lymphangioleiomyomatosis - low magnification|thumb|left]] | |||
[[File:Lymphangioleiomyomatose_CT.jpg|Lymphangioleiomyomatosis CT scan|thumb]] | |||
[[File:Diagnostic_Algorithm_for_Lymphangioleiomyomatosis.jpg|Diagnostic Algorithm for Lymphangioleiomyomatosis|thumb|left]] | |||
'''Lymphangioleiomyomatosis''' ('''LAM''') is a rare lung disease that typically affects women of childbearing age. In LAM, muscle cells that line the lungs' airways and blood vessels begin to multiply abnormally. These LAM cells form clusters and can cause blockages, leading to a variety of symptoms. | '''Lymphangioleiomyomatosis''' ('''LAM''') is a rare lung disease that typically affects women of childbearing age. In LAM, muscle cells that line the lungs' airways and blood vessels begin to multiply abnormally. These LAM cells form clusters and can cause blockages, leading to a variety of symptoms. | ||
== Symptoms == | == Symptoms == | ||
The symptoms of LAM can vary greatly from person to person. Some people may have no symptoms at all, while others may experience: | The symptoms of LAM can vary greatly from person to person. Some people may have no symptoms at all, while others may experience: | ||
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* [[Fatigue]] | * [[Fatigue]] | ||
* [[Wheezing]] | * [[Wheezing]] | ||
== Causes == | == Causes == | ||
The exact cause of LAM is unknown. However, it is believed to be related to mutations in the [[TSC1]] or [[TSC2]] genes. These genes are involved in controlling cell growth, and mutations can lead to uncontrolled growth and division of cells. | The exact cause of LAM is unknown. However, it is believed to be related to mutations in the [[TSC1]] or [[TSC2]] genes. These genes are involved in controlling cell growth, and mutations can lead to uncontrolled growth and division of cells. | ||
== Diagnosis == | == Diagnosis == | ||
Diagnosis of LAM can be challenging, as its symptoms are similar to those of other lung diseases. However, certain tests can help in the diagnosis: | Diagnosis of LAM can be challenging, as its symptoms are similar to those of other lung diseases. However, certain tests can help in the diagnosis: | ||
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* [[Biopsy]] | * [[Biopsy]] | ||
* [[Blood test]] | * [[Blood test]] | ||
== Treatment == | == Treatment == | ||
There is currently no cure for LAM, but treatments can help manage symptoms and slow the progression of the disease. These may include: | There is currently no cure for LAM, but treatments can help manage symptoms and slow the progression of the disease. These may include: | ||
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* [[Lung transplantation]] | * [[Lung transplantation]] | ||
* Medications such as [[sirolimus]] | * Medications such as [[sirolimus]] | ||
== Prognosis == | == Prognosis == | ||
The prognosis for individuals with LAM varies. Some people may remain stable for many years, while others may experience a rapid progression of the disease. | The prognosis for individuals with LAM varies. Some people may remain stable for many years, while others may experience a rapid progression of the disease. | ||
== See also == | == See also == | ||
* [[Tuberous sclerosis]] | * [[Tuberous sclerosis]] | ||
* [[Pulmonary fibrosis]] | * [[Pulmonary fibrosis]] | ||
* [[Pulmonary hypertension]] | * [[Pulmonary hypertension]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
[[Category:Lung disorders]] | [[Category:Lung disorders]] | ||
[[Category:Genetic disorders]] | [[Category:Genetic disorders]] | ||
{{stub}} | {{stub}} | ||
Latest revision as of 21:21, 12 April 2025
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC
| Lymphangioleiomyomatosis | |
|---|---|
| |
| Synonyms | LAM |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Shortness of breath, chest pain, cough, pneumothorax, chylothorax |
| Complications | Respiratory failure, pulmonary hypertension, kidney angiomyolipoma |
| Onset | Typically in women of childbearing age |
| Duration | Chronic |
| Types | N/A |
| Causes | Genetic mutation in the TSC1 or TSC2 genes |
| Risks | Female gender, tuberous sclerosis complex |
| Diagnosis | CT scan, lung biopsy, pulmonary function test |
| Differential diagnosis | Asthma, chronic obstructive pulmonary disease, interstitial lung disease |
| Prevention | N/A |
| Treatment | Sirolimus, everolimus, lung transplantation |
| Medication | Bronchodilators, hormonal therapy |
| Prognosis | Variable, can lead to progressive lung disease |
| Frequency | Rare, estimated 3-5 per million women |
| Deaths | Can be fatal if untreated |
Lymphangioleiomyomatosis (LAM) is a rare lung disease that typically affects women of childbearing age. In LAM, muscle cells that line the lungs' airways and blood vessels begin to multiply abnormally. These LAM cells form clusters and can cause blockages, leading to a variety of symptoms.
Symptoms[edit]
The symptoms of LAM can vary greatly from person to person. Some people may have no symptoms at all, while others may experience:
- Shortness of breath, especially during physical activity
- Chest pain
- Frequent cough, sometimes with blood (hemoptysis)
- Fatigue
- Wheezing
Causes[edit]
The exact cause of LAM is unknown. However, it is believed to be related to mutations in the TSC1 or TSC2 genes. These genes are involved in controlling cell growth, and mutations can lead to uncontrolled growth and division of cells.
Diagnosis[edit]
Diagnosis of LAM can be challenging, as its symptoms are similar to those of other lung diseases. However, certain tests can help in the diagnosis:
Treatment[edit]
There is currently no cure for LAM, but treatments can help manage symptoms and slow the progression of the disease. These may include:
- Bronchodilators
- Oxygen therapy
- Lung transplantation
- Medications such as sirolimus
Prognosis[edit]
The prognosis for individuals with LAM varies. Some people may remain stable for many years, while others may experience a rapid progression of the disease.
