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Angioid Streaks

Disease Details

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Description: Angioid streaks are irregular, jagged lines that radiate from the optic disc and are often associated with various systemic diseases, leading to changes in Bruch's membrane in the eye.
Type: Angioid streaks can be associated with genetic diseases, most notably Pseudoxanthoma Elasticum (PXE). PXE typically follows an autosomal recessive pattern of genetic transmission.
Signs And Symptoms: Angioid streaks are irregular, crack-like deformations in the Bruch's membrane, a layer of the eye. They can be associated with several systemic conditions, but often appear without any symptoms.

**Signs and Symptoms:**
1. **Visual changes**: Blurred vision, distorted vision, or a decrease in visual acuity.
2. **Dark streaks**: Usually radiate from the optic disc; these can be seen during a detailed eye examination.
3. **Subretinal hemorrhage**: This can occur and lead to vision loss.
4. **Metamorphopsia**: Distortion of vision where straight lines may appear wavy.

Nanotechnology is not typically discussed in relation to angioid streaks, so it's not applicable (nan).
Prognosis: The prognosis for angioid streaks can vary depending on the underlying cause and the presence of complications. Angioid streaks are associated with several systemic conditions such as pseudoxanthoma elasticum, Ehlers-Danlos syndrome, and Paget's disease.

For many individuals, angioid streaks remain stable without significant vision loss. However, some may experience complications such as choroidal neovascularization, which can lead to severe vision impairment if not properly managed. Regular monitoring by an eye specialist is crucial for early intervention and better outcomes.

Note: "nan" refers to "not a number" and appears extraneous in this context.
Onset: Angioid streaks typically manifest in adulthood, often between the ages of 20 and 40. In some cases, they may remain asymptomatic and be discovered incidentally during a routine eye examination.
Prevalence: The prevalence of angioid streaks is not well-defined in the general population, and specific numbers are not readily available. They are, however, associated with certain systemic conditions such as pseudoxanthoma elasticum, Ehlers-Danlos syndrome, and sickle cell disease, among others. The presence of these underlying conditions significantly increases the likelihood of developing angioid streaks.
Epidemiology: Angioid streaks are irregular, crack-like dehiscences in Bruch's membrane, which is a part of the eye's retina. These streaks often radiate outward from the optic disc and are typically associated with various systemic conditions, most notably pseudoxanthoma elasticum (PXE), but also Ehlers-Danlos syndrome, Paget's disease, and sickle cell disease.

Epidemiology:
- Angioid streaks are relatively rare, affecting a small percentage of the general population.
- They are more frequently observed in adults than in children, often being diagnosed in individuals during their middle age.
- The prevalence varies somewhat depending on the underlying associated systemic condition.
- There does not appear to be a significant predilection for any specific gender or ethnicity.
Intractability: Angioid streaks, which are breaks in Bruch's membrane of the retina, are typically associated with systemic conditions such as pseudoxanthoma elasticum, Ehlers-Danlos syndrome, or Paget's disease. While the underlying systemic conditions may be challenging to cure, the ocular aspect of angioid streaks themselves is generally considered intractable in terms of complete elimination. However, regular monitoring and treatments can manage complications like choroidal neovascularization associated with angioid streaks.
Disease Severity: Angioid streaks are irregular, crack-like deformations in the Bruch's membrane of the eye, associated with various systemic diseases. The severity can vary depending on the underlying condition. While some patients may have minimal visual impairment, others might experience significant vision loss due to complications such as choroidal neovascularization or macular degeneration. The severity is generally assessed through regular ophthalmologic examinations and monitoring of any associated systemic conditions.
Healthcare Professionals: Disease Ontology ID - DOID:13401
Pathophysiology: Angioid streaks are irregular, crack-like deformations in Bruch's membrane, an important layer of the retina. They are commonly associated with a variety of systemic diseases, the most notable being Pseudoxanthoma Elasticum (PXE), Ehlers-Danlos Syndrome, Paget's Disease, and Sickle Cell Disease. The pathophysiology is primarily characterized by the calcification and subsequent fragmentation of the elastic tissue within Bruch's membrane, leading to the formation of these streaks. This weakening of the structural integrity of the membrane makes it susceptible to breaks and cracks, visible as reddish-brown to gray linear streaks radiating from the optic disc. In advanced cases, these changes can lead to complications such as choroidal neovascularization, potentially resulting in significant vision loss.
Carrier Status: Angioid streaks are a manifestation seen in several genetically inherited disorders, but there is no specific "carrier status" for angioid streaks themselves. Rather, they are typically associated with systemic conditions such as pseudoxanthoma elasticum, Ehlers-Danlos syndrome, and sickle cell disease.
Mechanism: Angioid streaks are irregular, crack-like deformations in Bruch's membrane, which is a part of the retina. They are often associated with systemic diseases such as pseudoxanthoma elasticum (PXE), Ehlers-Danlos syndrome, and Paget's disease.

**Mechanism:**
The primary mechanism involves the calcification and subsequent brittleness of Bruch's membrane. When this membrane becomes brittle, it can develop cracks or breaks that appear as streaks on retinal imaging. These streaks can allow blood vessels from the choroid to grow into the retina, leading to potential complications like choroidal neovascularization, which can threaten vision.

**Molecular Mechanisms:**
1. **Collagen and Elastin Defects:** In conditions like pseudoxanthoma elasticum, mutations in the ABCC6 gene impair the regulation of extracellular matrix minerals, leading to calcification. This affects the structural integrity of collagen and elastin in Bruch's membrane.
2. **Impaired Mineralization:** Abnormal mineralization processes occurring due to defective biomolecules affect the strength and flexibility of Bruch's membrane, creating predisposition to breaks.
3. **Inflammatory Processes:** Chronic low-grade inflammation can contribute to the malfunctioning and weakening of the membrane's structural proteins.

These molecular disruptions ultimately compromise the structural integrity of Bruch's membrane, making it susceptible to the formation of angioid streaks.
Treatment: Angioid streaks are irregular, crack-like deformations in the Bruch's membrane of the eye, often associated with various systemic conditions, including pseudoxanthoma elasticum and Ehlers-Danlos syndrome. Treatment focuses on managing complications, particularly choroidal neovascularization (CNV), which can lead to vision loss.

1. **Anti-VEGF Therapy:** Intravitreal injections of anti-vascular endothelial growth factor (anti-VEGF) agents such as ranibizumab or bevacizumab are commonly used to treat CNV and prevent further vision deterioration.
2. **Laser Photocoagulation:** This may be used in some cases to seal abnormal blood vessels and prevent further damage.
3. **Photodynamic Therapy (PDT):** This involves using a photosensitizing drug and a laser to target abnormal blood vessels in the eye.

Regular monitoring by an ophthalmologist is essential to detect and manage complications early.
Compassionate Use Treatment: Angioid streaks are irregular streaks in the retina that are often associated with systemic conditions like pseudoxanthoma elasticum. While there is no specific established treatment for angioid streaks themselves, the following off-label or experimental treatments might be considered to manage complications, such as choroidal neovascularization (CNV):

1. **Anti-VEGF Therapy**: Drugs such as ranibizumab (Lucentis) and bevacizumab (Avastin) are commonly used off-label to manage CNV, a serious complication of angioid streaks. These medications inhibit vascular endothelial growth factor (VEGF) to reduce abnormal blood vessel growth.

2. **Photodynamic Therapy (PDT)**: This treatment involves using a photosensitizing agent along with laser light to destroy abnormal blood vessels. It's less commonly used now due to the effectiveness of anti-VEGF treatments but can still be considered in specific cases.

3. **Laser Photocoagulation**: Argon laser photocoagulation has been used in the past to treat CNV secondary to angioid streaks, though it is less favored due to potential complications like vision loss.

4. **Systemic Treatments**: For underlying conditions associated with angioid streaks, appropriate management may indirectly improve retinal health. For example, addressing systemic diseases like pseudoxanthoma elasticum or sickle cell disease may help in overall disease management.

5. **Experimental Treatments**: Clinical trials exploring new therapeutic agents or gene therapy approaches may offer potential future treatments. Participation in such trials can be an option for some patients.

Consultation with a retinal specialist is essential to determine the most appropriate treatment plan based on the individual's specific condition and associated risks.
Lifestyle Recommendations: Angioid streaks are irregular, crack-like deformations in Bruch's membrane, typically affecting the eyes and potentially leading to vision problems. While they are associated with several systemic conditions, lifestyle recommendations generally focus on:

1. **Regular Eye Exams**: To monitor any changes or progression in the streaks, it's crucial to have frequent check-ups with an ophthalmologist.

2. **Protect the Eyes**: Wearing protective eyewear can help prevent trauma, which can exacerbate the condition.

3. **Maintain Systemic Health**: Conditions like pseudoxanthoma elasticum and Ehlers-Danlos syndrome can be associated with angioid streaks. Managing any underlying conditions through medication, diet, and lifestyle changes is essential.

4. **Avoid High-impact Activities**: To prevent trauma to the eyes, avoid activities that carry a high risk of blows or pressure to the head and eyes.

5. **Healthy Diet**: A balanced diet rich in vitamins and minerals supports overall eye health.

6. **No Smoking**: Smoking can exacerbate many eye conditions, including those associated with angioid streaks. Avoiding smoking is beneficial.

While these recommendations can help manage the condition, personalized advice from healthcare providers is important for optimal management.
Medication: Angioid streaks, which are irregular lines in the retina often associated with various systemic diseases, do not have a specific medication for treatment. Management focuses on monitoring and addressing underlying conditions (such as pseudoxanthoma elasticum, Ehlers-Danlos syndrome, or Paget's disease) and complications like choroidal neovascularization. In cases of choroidal neovascularization, treatments might include anti-VEGF (vascular endothelial growth factor) injections to reduce abnormal blood vessel growth and leakage. Regular eye exams and multidisciplinary care tailored to individual patient needs are essential.
Repurposable Drugs: No current repurposable drugs are specifically approved for the treatment of angioid streaks. Angioid streaks are typically associated with conditions like pseudoxanthoma elasticum, Ehlers-Danlos syndrome, and other systemic diseases. Management focuses on treating complications such as choroidal neovascularization, for which anti-VEGF (vascular endothelial growth factor) treatments originally designed for age-related macular degeneration may be used. Regular monitoring and addressing any systemic conditions associated with angioid streaks are essential.
Metabolites: Angioid streaks are irregular lines that radiate from the optic disc of the eye, which are often associated with various systemic conditions such as pseudoxanthoma elasticum, Ehlers-Danlos syndrome, and Paget's disease. They affect Bruch's membrane, one of the layers of the retina.

In terms of metabolites, there is no specific direct association between angioid streaks and particular metabolites. Instead, evaluations focus on the underlying systemic conditions that may contribute to the development of angioid streaks, such as lipid metabolism disorders in pseudoxanthoma elasticum. Blood tests and imaging can be used to identify relevant underlying systemic conditions, and management strategies typically address the primary disorder rather than the streaks themselves. Regular monitoring by an ophthalmologist is recommended to manage potential complications, like choroidal neovascularization, which can impact vision.
Nutraceuticals: Currently, there is limited evidence to support specific nutraceuticals for the treatment or prevention of angioid streaks. Angioid streaks are primarily associated with systemic conditions like pseudoxanthoma elasticum and other connective tissue disorders. It's important for patients to consult healthcare providers for management strategies tailored to their specific condition.
Peptides: Angioid streaks are irregular, jagged lines that can appear in the retina of the eye. They are not directly associated with peptides. The cause of angioid streaks is often related to systemic conditions that affect the integrity of the Bruch's membrane in the eye, such as pseudoxanthoma elasticum, Ehlers-Danlos syndrome, Paget’s disease, and sickle cell disease. There is no established connection between angioid streaks and nanoparticles (nan).