Cystic Lung Disease Flashcards
What is the condition characterized by cystic lung disease (9)
- Lymphangioleiomyomatosis
- Pulmonary Langerhans Cell Histiocytosis
- Lymphoid Interstitial Pneumonia
- Follicular Bronchitis
- Amyloidosis
- Light Chain Deposition Disease
- Birt-Hogg- Dube Syndrome
- Desquamative Interstitial Pneumonia
- Cystic Pulmonary Metastatic Disease
What is Lymphangiomyomatosis (LAM)?
A rare lung disease that primarily affects women of childbearing age
Characterized by smooth muscle proliferation in the pulmonary interstitium.
What is the main demographic affected by Lymphangiomyomatosis?
Women of childbearing age
Often associated with tuberous sclerosis complex.
List key clinical characteristics of Lymphangiomyomatosis.
- Chylothorax
- Angiomyolipomas
- Spontaneous pneumothorax
These manifestations are linked to the disease’s progression.
What is the pathophysiology of Lymphangiomyomatosis?
Smooth muscle proliferation in pulmonary interstitium
Involves blood vessels, airways, lymphatics, and alveolar septa.
What is Pulmonary Langerhans cell histiocytosis (PLCH) commonly associated with?
Smoking
Affects young adults and can lead to pulmonary hypertension.
What are the HRCT findings for Pulmonary Langerhans cell histiocytosis?
- Upper lobe predominant centriloblar nodules
- Sparing of the costophrenic angle
- Round or irregular cysts (bilobed, clover leaf, bizzare shapes)
What is Desquamative Interstitial Pneumonia (DIP)?
A lung disease characterized by the accumulation of macrophages in the alveoli
Often associated with smoking.
What is the relationship between Lymphoid Interstitial Pneumonia (LIP) and autoimmune diseases?
LIP is often associated with autoimmune diseases such as:
- Sjogren Disease
- Primary Biliary Cirrhosis
- SLE
- AIDS
- Autoimmune Thyroid Disease
Conditions include primary biliary cirrhosis and systemic lupus erythematosus.
What is the typical size of cysts in Lymphangiomyomatosis?
Cysts can range from 2 to 30 mm
Surrounding normal lung tissue is often observed.
True or False: Follicular Bronchiolitis is primarily a neoplastic process.
False
It is a lymphoproliferative disorder.
What is the imaging characteristic of cystic lung diseases like LAM and PLCH?
Presence of thin-walled cysts and irregular nodules
Cysts may also show a variety of sizes and shapes.
What is the significance of the term ‘angiomyolipomas’ in the context of Lymphangiomyomatosis?
These are benign tumors commonly found in patients with LAM
They often arise in the kidneys.
Fill in the blank: The presence of _____ is a common finding in Pulmonary Langerhans cell histiocytosis.
Langerhans cells
These cells are a type of immune system cell involved in the disease.
What are common pulmonary complications of Lymphangiomyomatosis?
- Spontaneous pneumothorax
- Chylothorax
- Pulmonary hypertension
These complications significantly affect the patient’s health.
What is the typical presentation of cysts in DIP?
Small cysts surrounded by ground glass opacities associated with centrilobular emphysema, peripheral cyst location.
Often seen in imaging studies.
What is the role of Langerhans cells in Pulmonary Langerhans cell histiocytosis?
They are antigen-presenting cells (APCs) involved in the immune response
Their proliferation leads to airway inflammation.
What is amyloidosis?
A condition characterized by the deposition of amyloid proteins in various tissues and organs
Amyloidosis can lead to organ dysfunction and is often associated with conditions like multiple myeloma and rheumatoid arthritis.
What are light chain deposits associated with?
Conditions like multiple myeloma and macroglobulinemia
These conditions involve the production of abnormal proteins that can deposit in tissues.
List some secondary conditions that can lead to amyloidosis.
- Rheumatoid arthritis
- Tuberculosis
- Crohn’s disease
- Cystic fibrosis
- Mediterranean fever
These conditions can trigger the abnormal protein deposition characteristic of amyloidosis.
What are the common HRCT findings in patients with amyloidosis?
*Interlobular septal thickening
*Honeycombing
* Ground glass opacities
* Lymphadenopathy
These findings can indicate pulmonary involvement in amyloidosis.
What is Birt-Hogg-Dubé syndrome?
An autosomal dominant disorder characterized by fibrofolliculomas
It affects the skin and is also associated with an increased risk of certain cancers.
What is Desquamative Interstitial Pneumonia (DIP)?
A form of interstitial lung disease often associated with smoking
DIP is characterized by the accumulation of macrophages in the alveoli.
What are some features of pulmonary metastatic disease?
- Thin-walled cysts of various sizes
These features can indicate the presence of metastatic cancer in the lungs.
True or False: Lymphadenopathy can be a feature of amyloidosis.
True
Lymphadenopathy may occur due to the systemic effects of amyloid deposition.
What types of tumors can lead to pulmonary complications in amyloidosis?
- Sarcomas
- Carcinomas
These tumors can contribute to lung pathology and complicate the clinical picture.
Fill in the blank: Amyloidosis may lead to _____ opacities on imaging.
ground-glass
Ground-glass opacities indicate areas of inflammation or fluid in the lungs.
