retinal vasculitis

ICD-10 Codes

Related ICD-10:

Description

Retinal vasculitis refers to an inflammatory condition that affects the blood vessels in the retina, which is a critical part of the eye responsible for vision.

Characteristics of Retinal Vasculitis

  • Inflammation of the retinal arteries and/or veins [3]
  • Vascular sheathing (swelling of the blood vessel walls) [3]
  • Presence of vitreous cells (abnormal cells in the gel-like substance that fills the center of the eye) [3]
  • Cotton-wool spots (small, fluffy white patches on the retina) [3]
  • Intraretinal hemorrhages (bleeding within the retina) [3]

Causes and Associations

  • Infectious diseases such as toxoplasmosis, varicella zoster, herpes simplex, cytomegalovirus, and human T-cell lymphoma virus type 1 [1]
  • Systemic vasculitis conditions like Behçet's disease, sarcoidosis, multiple sclerosis, temporal arteritis, polyarteritis nodosa, and granulomatosis with polyangiitis [4, 8]
  • Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN), a rare inflammatory condition characterized by abnormal widening of the arteries and optic nerve inflammation [5]

Complications

  • Macular edema (swelling of the macula, which is responsible for central vision) [3]
  • Vascular occlusion (blockage of blood vessels in the retina) [3]
  • Retinal ischemia (reduced blood flow to the retina) [3]
  • Neovascularization (formation of new, fragile blood vessels in the retina) [3]

These characteristics and complications can lead to significant vision loss if left untreated or undertreated.

Additional Characteristics

  • or veins
  • Vascular sheathing (swelling of the blood vessel walls)
  • Presence of vitreous cells (abnormal cells in the gel-like substance that fills the center of the eye)
  • Cotton-wool spots (small, fluffy white patches on the retina)
  • Intraretinal hemorrhages (bleeding within the retina)
  • Macular edema (swelling of the macula, which is responsible for central vision)
  • Vascular occlusion (blockage of blood vessels in the retina)
  • Retinal ischemia (reduced blood flow to the retina)
  • Neovascularization (formation of new, fragile blood vessels in the retina)

Signs and Symptoms

Common Signs and Symptoms of Retinal Vasculitis

Retinal vasculitis, an inflammation of the blood vessels in the retina, can manifest in various ways. The symptoms and signs of this condition can help point a physician toward the underlying cause.

  • Perivascular Sheathing: A white cuff made up of inflammatory cells that appears around the blood vessels is one of the most common signs of retinal vasculitis [1][10].
  • Visual Changes:
    • Blurred Vision: Often described as looking through a "fog" or "haze," blurred vision is an early and common symptom [11].
    • Floaters: Dark spots or "cobwebs" floating in the field of vision, particularly against bright backgrounds, can be noticed by patients [11].
  • Decreased Visual Acuity: Vasculitis near the macula can cause loss of visual acuity [12].
  • Asymptomatic Cases: Vasculitis of peripheral retinal vessels may be asymptomatic [12].

Other symptoms and signs associated with retinal vasculitis include:

  • Painless Decrease in Vision [8]
  • Blind Spot [8]
  • Redness and Eye Pain [5]
  • Increased Sensitivity to Light [5]

It is essential to note that the prognosis of retinal vasculitis can vary depending on the stage at presentation and the underlying cause of the disease. Vision loss can occur, especially when the central retina is involved.

References:

[1] Context result 1 [5] Context result 5 [8] Context result 8 [10] Context result 10 [11] Context result 11 [12] Context result 12

Additional Symptoms

  • Floaters
  • Decreased Visual Acuity
  • Increased Sensitivity to Light
  • Perivascular Sheathing
  • Painless Decrease in Vision
  • Redness and Eye Pain
  • blind spot
  • blurred vision

Diagnostic Tests

Retinal vasculitis can be diagnosed through a combination of clinical assessment, ophthalmic imaging, and tailored laboratory investigations.

Clinical Examination The diagnosis of retinal vasculitis relies heavily on clinical examination by an eye care provider. A thorough examination of the retina is performed to identify any signs of inflammation or vascular damage [1]. The clinical examination may reveal symptoms such as pain, redness, and swelling in the affected eye.

Ophthalmic Imaging In addition to clinical examination, ophthalmic imaging plays a crucial role in diagnosing retinal vasculitis. Fundus fluorescein angiography (FFA) is a commonly used imaging technique that helps evaluate the presence and extent of retinal vasculitis [2]. FFA involves injecting a fluorescent dye into the bloodstream, which highlights the blood vessels in the retina.

Laboratory Investigations Supportive laboratory tests are also essential in diagnosing retinal vasculitis. These may include positive findings on laboratory tests such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), which indicate inflammation [3]. Other laboratory investigations may be performed to rule out other conditions that may mimic retinal vasculitis.

Other Diagnostic Tests Fluorescein angiography is used to evaluate the presence and extent of retinal vasculitis, but cannot reliably differentiate between vascular damage from other causes [4]. Indocyanine green angiography (ICGA) may also be used to evaluate the retina and detect any signs of inflammation or vascular damage.

Specialized Care While retinal vasculitis can be detected by any eye care provider, further testing and treatment generally requires a uveitis specialist. These doctors specialize in the diagnosis and management of inflammatory conditions affecting the eye [5].

References: [1] Context 4 [2] Context 2 [3] Context 9 [4] Context 8 [5] Context 5

Additional Diagnostic Tests

  • Clinical Examination
  • Fundus fluorescein angiography (FFA)
  • Laboratory tests (ESR, CRP)
  • Indocyanine green angiography (ICGA)

Treatment

Retinal vasculitis can be treated with various medications, depending on its underlying cause and severity. Here are some common drug treatments for retinal vasculitis:

  • Corticosteroids: These are often the first line of treatment for non-infectious cases of retinal vasculitis. They can be administered systemically (oral or intravenous) or locally (periocular or intravitreal). [2][3]
  • Antibiotics and antivirals: For infectious causes, such as toxoplasmosis or herpes simplex, antibiotics and antiviral medications are used to treat the underlying infection. [1]
  • Immunosuppressive medications: These may be necessary for non-infectious cases of retinal vasculitis that do not respond to corticosteroids alone. Examples include azathioprine, cyclosporine, and rituximab. [4][5][6]
  • Anti-TB agents: For retinal vasculitis caused by tuberculosis (TB), anti-TB medications such as isoniazid, rifampin, and pyrazinamide are used in combination with corticosteroids. [7][9]
  • Avacopan: This medication has been approved for the treatment of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), two types of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. [10]

It's worth noting that the choice of medication depends on the underlying cause of retinal vasculitis, as well as the severity and duration of symptoms. In some cases, a combination of medications may be necessary to achieve optimal results.

References:

[1] Context result 1 [2] Context result 2 [3] Context result 6 [4] Context result 5 [5] Context result 7 [6] Context result 8 [7] Context result 9 [10] Context result 10

Recommended Medications

  • Corticosteroids
  • Immunosuppressive medications
  • Avacopan
  • Antibiotics and antivirals
  • Anti-TB agents

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Differential Diagnoses of Retinal Vasculitis

Retinal vasculitis, a sight-threatening inflammatory eye condition, can be caused by various ocular and systemic etiologies. The differential diagnoses for retinal vasculitis are numerous and include:

  • Infectious causes: Toxoplasmosis, tuberculosis (TB), syphilis, herpes viral infections, Bartonella, Brucellosis, Lyme's disease, Leptospirosis [5][6]
  • Systemic diseases: Behcet's disease (BD), multiple sclerosis (MS), systemic lupus erythematosus (SLE) [9]
  • Idiopathic retinal vasculitis: A subgroup of patients without any underlying ocular or systemic cause [1][15]

Other causes

  • Retinal vasculitis can also be associated with intermediate uveitis, posterior uveitis, or panuveitis [7]
  • It is a sight-threatening disease due to complications from macular edema, vascular occlusion, retinal ischemia, and neovascularization [2]

Diagnostic approach

The laboratory work-up of a patient with retinal vasculitis should be based on a differential diagnosis derived from a detailed history, review of systems, and physical examination. If the patient's medical history, review of systems, or ocular examination suggests an underlying systemic disease, then the diagnostic work-up should be tailored accordingly [12][14]

References

[1] The differential diagnoses of retinal vasculitis have been listed in Table 2. [2] Retinal vasculitis is a diagnosis of inflammatory changes of the retinal arteries and/or veins consisting of vascular sheathing, vitreous cells, cotton-wool spots, and intraretinal hemorrhages. [5] The most common cause of infectious retinal vasculitis is toxoplasmosis, followed by tuberculosis (TB), syphilis, and herpes viral infections. [6] Retinal vasculitis can be caused by several bacterial agents including tuberculosis, syphilis, Lyme's disease, Brucellosis, Bartonella, and Leptospirosis. [7] Retinal vasculitis is characterized by perivascular infiltration of lymphocytes. It can be observed in intermediate uveitis, posterior uveitis, or panuveitis. [9] Detection of retinal vasculitis is made clinically, and confirmed with the help of fundus fluorescein angiography. [12] The laboratory work-up of a patient with retinal vasculitis should be based on a differential diagnosis derived from a detailed history, review of systems, and physical examination. [14] Retinal vasculitis is a sight-threatening inflammatory eye condition that involves the retinal vessels. Detection of retinal vasculitis is made clinically, and confirmed with the help of fundus fluorescein angiography.

Additional Information

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