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Classification of uveitis

  • Writer: Nelson Santos
    Nelson Santos
  • Mar 30, 2020
  • 8 min read

Updated: Apr 3, 2022

The criteria for classifying uveitis is very broad and can be a bit confusing, but it is essential to manage the case as well as possible and choose the most appropriate treatment. Medical practice currently classifies uveitis according to the following main categories:


1. Anatomical location of inflammation

2. Affected tissues

3. Presence of keratic precipitates

4. Duration and severity

5. How symptoms develop

6. Cause (etiology)

7. Association with systemic diseases


Uveitis can also be classified according to this additional criteria:


8. Laterality: unilateral or bilateral

9. Age of initial inflammatory episode

10. Immunity status

11. Endogenous or exogenous



Anterior uveitis


The inflammation is mostly in the anterior chamber. There may also be inflammation in the iris and anterior part of the ciliary body


Associated symptoms


Anterior uveitis - pain, redness (hyperemia), sensitivity to light (photophobia), some blurring of vision.


Chronic anterior uveitis - produces no symptoms


Intermediate uveitis


The inflammation is mostly found in the vitreous. Inflammation may also occur in the pars plana and anterior retina. The most common complications associated with intermediate uveitis are inflammation of the macula and/or optic nerve.


Associated symptoms


Floaters, loss of central vision, altered or decreased vision.


Posterior uveitis


The inflammation is mostly found in the choroid and/or retina. Occasionally, it may also involve the optic disc, optic nerve, blood vessels and posterior vitreous. In its strict definition, posterior uveitis is characterized by the presence of one or more lesions in the retina or choroid. This does not include structural complications such as macular edema, vasculitis or optic nerve inflammation.


Associated symptoms


Stationary dark spots in the field of vision (scotomas), loss of central vision, decreased vision. In some cases, posterior uveitis does not produce symptoms if lesions in the retina and/or choroid do not affect the visual field.


Panuveitis


All three parts of the uvea are inflamed (iris, ciliary body and choroid). It is not unusual to also present retinal and/or optic nerve involvement.


Associated symptoms


Pain, redness (hyperemia), sensitivity to light (photophobia), floaters, spots in the field of vision (scotomas), loss of central vision.


Uveitis will never manifest the same way in all patients because its clinical presentation is varied. For this reason, keep in mind that there may be some degree of variation in your case.


Affected tissues


All parts of the eye are composed of tissues and structures with specific functions. Therefore, uveitis is further classified according to the tissues that are inflamed:


Anterior uveitis:

Iritis

Iridocyclitis


Intermediate uveitis:

Vitritis

Hyalitis

Posterior cyclitis

Pars planitis


Posterior uveitis:

Choroiditis (focal, multifocal or diffuse).

Retinitis

Chorioretinitis

Retinochoroiditis

Neuroretinitis

Retinovasculitis

Papillitis


Panuveitis:

Inflammation may be present in the anterior cavity, vitreous, retina or choroid


Presence of keratic cells or precipitates


The presence of keratic cells or precipitates may offer clues as to the possible cause of uveitis. However, it is not a definitive criterion since the presence of these keratic cells or precipitates has shown variability during the course of recurrent inflammatory episodes. For this reason, rather than a pathologic or diagnostic description, it is a clinical description: it helps to characterize the clinical picture.


The presence of keratic cells or precipitates is counted to measure the degree of inflammation and the effectiveness of treatments. Keratotic precipitates are cells associated with the inflammatory process that may be present in the anterior chamber or vitreous, depending on the location of the inflammation. Anterior uveitis is classified as granulomatous or non-granulomatous depending on the accumulation of these precipitates on the posterior surface of the cornea (corneal endothelium) or iris.


Granulomatous

It presents giant cells, epithelial cells, mutton-fat keratic precipitates and chorioretinal lesions. The appearance of these cells is often described as oily. Granulomatous uveitis is mostly associated with systemic disease. Iris nodules (Koeppe or Busacca) are granulomatous cells that occur in cases of syphilis and sarcoidosis, but not in cases of ocular herpes and sympathetic ophthalmia.


Some causes associated with granulomatous uveitis are:

Non-granulomatous

It is characterized by the presence of lymphocytes,plasma cells or fine keratic precipitates. The appearance of these cells is often described as "snowballs" or "starry skies". Non-granulomatous uveitis is statistically associated with idiopathic cases, but is also caused by some diseases.


Some causes associated with non-granulomatous uveitis are:

Granulomatous etiologies may masquerade as non-granulomatous processes, but non-granulomatous etiologies never present as granulomatous. An example of this is that syphilis and tuberculosis may present with non-granulomatous cells in their early stage, but end up developing granulomatous cells during the course of the disease.


On the other hand, intermediate uveitis usually presents cells and proteins in the vitreous chamber. These cells and proteins may be seen as white exudates (snowballs) in the vitreous chamber. Cases of severe inflammation may present deposits of these white exudates at the bottom of the vitreous chamber (snow banks).


Duration and severity


Uveitis is a medical emergency because the structures of the eyes are very delicate and the presence of inflammation could jeopardize the patient's vision. Sometimes it is necessary to take urgent measures, depending on the tissues and structures that are affected, how frequent the symptoms are and the possible association with systemic diseases. For example, posterior uveitis is associated with more severe complications than anterior or intermediate uveitis. Therefore, treatment for posterior uveitis should be promptly implemented to avoid the risk of vision loss. However, the reactivation of uveitis depends primarily on its cause and how well patients respond to the chosen treatment.


The duration and severity of inflammation is classified according to three main categories: acute, recurrent or chronic. Acute uveitis is characterized by the sudden onset of symptoms, but they usually last for a short period of time under appropriate anti-inflammatory treatment. On the other hand, if a new inflammatory episode occurs after treatment has been completed, the uveitis is referred to as recurrent. Finally, the occurrence of ocular inflammation as soon as treatment ends, or even while the patient is on medication, is indicative of chronic uveitis.


Some causes are associated with more aggressive courses than others. Therefore, identifying the cause of uveitis can help elucidate the prognosis of the case and the duration of treatment. Acute diseases are those that can be remedied under short-term treatment. In contrast, chronic diseases require long-term treatment and impact patients' quality of life. Recognizing the course of the disease, especially in chronic cases, is important for patients to be able to adjust to the new living conditions that chronic diseases bring. In addition, it allows the development of proactive skills for the management of their disease and the prevention of complications.


Description of how symptoms may start to develop


The symptoms associated with acute anterior uveitis usually appear suddenly and force patients to go to the emergency room, as they are often extremely uncomfortable and painful (pain, photophobia, redness of the eye). In contrast, despite the severe complications that chronic anterior uveitis may cause, it is characterized by its insidious course: the mild symptoms it produces are not recognized by patients. As a result, they often do not seek help promptly and it is only diagnosed when serious complications have already arisen or during a routine eye examination. People who present symptoms associated with intermediate and posterior uveitis tend to postpone a visit to the doctor a little longer. These symptoms, although uncomfortable, are usually not accompanied by pain and include: blurred vision, spots in the field of vision, floaters, decreased or loss of vision, general vision changes, etc. These symptoms may develop suddenly or gradually, but most often the initial inflammatory episodes are sudden in nature. Intermediate and posterior uveitis should be treated promptly, even when painless, as they may cause other ocular complications. For this reason, it is extremely important to seek medical attention if symptoms occur. Any unusual ocular symptom is a compelling reason to be evaluated by an ophthalmologist, even if there is no pain or major discomfort.


Cause (etiology)


The exact cause of uveitis is never found in most cases. For this reason, the most important thing to do during this process is to classify them as infectious or non-infectious. This allows prompt administration of appropriate treatment and reduces the risk of ocular complications


Infectious uveitis: it may be caused by bacteria, virus, fungus or parasite.


Non-infectious uveitis: it could be idiopathic, traumatic in origin, caused by ocular surgery, associated with an autoimmune disease with systemic or local involvement. Although unusual, uveitis could also be caused by cancer, medication or tattoos.


Drugs that can induce uveitis include: rifabutin, etanercept, bisphosphonates, cidofovir, sulfonamides, latanoprost.


Tattoos on any part of the body can also cause uveitis. Especially those that were done in the past six to twelve months or so. They may present inflammation, induration, irritation, itching or itching along with uveitis. It is extremely important to rule out other ocular syndromes that may masquerade as uveitis, but are not inflammatory diseases. These syndromes are more common in pediatric patients than in adults. Some of the masking syndromes are:


Neoplastic diseases:

Retinoblastoma

Malignant uveal melanoma

Metastasis

Leukemia

Lymphoma

Reticulum sarcoma


Non-neoplastic diseases:

Retinitis pigmentosa

Intraocular foreign body

Juvenile xanthogranuloma

Retinal detachment

Pigmentary dispersion syndrome


Your ophthalmologist sub-specialized in ocular immunology and uveitis will be the person in charge of ruling out the possible main causes and recommending the most appropriate treatment.


Association with systemic diseases


In certain cases, uveitis may be caused by a systemic disease; that is, a disease that affects several organs of the body. This could be an infection or autoimmune disease. Your ophthalmologist will be in charge of your eye health, but if there is a possibility of an association with a systemic disease, they may refer you to another medical specialist.


Non-infectious uveitis may be associated with systemic autoimmune diseases. These types of diseases are usually of rheumatic, gastrointestinal or neurological origin. Because of the large connective tissue composition of the eyes, diseases that affect connective tissue elsewhere in the body may also affect the eyes. Similarly, diseases of the gastrointestinal or nervous system may also cause ocular symptoms and manifestations. In many cases of non-infectious uveitis, patients do NOT report symptoms in other parts of the body nor have they been previously diagnosed with any autoimmune disease. For this reason, it is not unusual for a first episode of uveitis to be the first manifestation of autoimmunity for many patients and for them to subsequently begin to develop the other symptoms associated with the systemic autoimmune disease causing their uveitis. One such example is the case of Jack Osborne, who was diagnosed with multiple sclerosis after being evaluated for changes in his vision. Click HERE to watch video.


The review of all body systems is an important aspect to establish a differential diagnosis in case there is a possibility of association with a systemic disease. Certain signs of particular importance are:


General symptoms


  • General malaise

  • Dropping objects unintentionally

  • Any of the following sensations in some parts of the body, especially in the extremities and/or face: numbness, paralysis, tingling, "pins and needles", hot/cold, pressure, etc.

  • Fainting

  • Seizures

  • Fatigue or excessive tiredness

  • Dizziness or vertigo

  • Loss of appetite

  • Unintentional weight gain or loss

  • Recurrent or persistent fever

  • Cold or night sweats

  • Inflammation of lymph nodes

  • Loss of balance and/or coordination

  • Sensitivity to sunlight or artificial light

  • Head symptoms

  • Thinning or loss of hair

  • Loss of pigment in the hair (white patches)

  • Severe or frequent headache

  • Scalp irritation

  • Neck pain or stiffness


Auditory symptoms


  • Tinnitus or "ringing in the ears"

  • Hearing impairment or loss

  • Severe or recurrent ear infection

  • Inflammation of the earlobes

  • Nose and mouth symptoms

  • Nose and mouth symptoms

  • Dry eyes and dry mouth

  • Recurrent or severe nose bleeds

  • Sinusitis, nasal congestion and/or nosebleed

  • Infection in teeth or gums

  • Inflammation of the gums


Cardiovascular symptoms


  • Chest pain

  • Inflammation or swelling of the legs

  • Easy or frequent bruising

  • Easy or frequent bleeding


Respiratory symptoms


  • Frequent or severe colds

  • Recurrent or chronic cough

  • Coughing up blood

  • Recent viral infection

  • Asthma

  • Breathing difficulties or shortness of breath

  • Bone and joint symptoms

  • Pain, stiffness or swelling in the joints

  • Back pain

  • Low back pain

  • Back pain when sleeping or waking up

  • Muscle pain


Skin symptoms


  • Ulcers in mucocutaneous areas of the body: mouth (aphthous ulcers), nose, genital and/or perianal ulcers.

  • Decreased or loss of pigment in the skin or hair: vitiligo or poliosis

  • Skin rashes, irritation or rashes

  • Skin ulcers

  • Itchy skin

  • Acneiform lesions on the face or body

  • Easy sunstroke on exposure to sunlight (photosensitivity)

  • Inflammation of the skin of the legs: superficial thrombophlebitis or erythema nodosum.

  • Pain in the fingers or toes when exposed to the cold

  • Change in color of fingers or toes when exposed to the cold

  • Yellowish skin


Gastrointestinal symptoms


  • Indigestion or gastrointestinal problems

  • Difficulty swallowing

  • Constipation

  • Recurrent or severe diarrhea

  • Blood in the stool

  • Ulcers in the digestive tract: mouth, stomach, intestines, anus

  • Indigestion or gastrointestinal problems

  • Incontinence


Genitourinary symptoms


  • Renal problems

  • Urinary incontinence

  • Blood in urine

  • Urethral discharge

  • Genital ulcers

  • Prostatitis

  • Testicular pain (epididymitis)


Other


  • Risk of exposure to sexually transmitted infections

  • Risk of exposure to tuberculosis

  • Risk of exposure to human immunodeficiency virus (HIV)

  • Ingestion of raw meats or undrinkable water

  • Contact with aniamialrs: edogst, catis, horrses or cc:cr:cricriteria:

  • Insect bites or tick bites

  • Recent trips to foreign countries


Uveitis can also be classified according to this additional criteria


Laterality: unilateral or bilateral

Age of initial inflammatory episode

Patient immunity status

Endogenous or exogenous







 
 
 

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