Overview of papillitis

Papillitis, also known as anterior optic neuritis, is an eye infection found in the optic disc structure of the eye, which contains the optic nerve that allows you to see. It can occur on its own or as a symptom of another eye-related condition.¹ However, it is important to note that even though these conditions have eye and eyesight involvement, it is possible for people with these diagnoses not to experience papillitis.

It is unclear what causes papillitis to occur, although it is assumed that it can be due to the following:

• Infection
• Inflammation
• Tissue breakdown
• Poor blood flow ¹

Signs and symptoms

Papillitis is considered to be a physical manifestation of a variety of conditions. However, this infection has its own set of signs and symptoms that can appear in either one or both eyes, such as:

• Eye tissue necrosis (the death of body tissue)
• Edema (swelling caused by a buildup of excess fluid in the body’s tissues)
• Hemorrhages
• Vision loss¹
• Eye pain (in some cases)²

Diagnosis

A diagnosis of papillitis is made by checking for the signs and symptoms listed above during a physical exam. People who may have this condition have their eyes checked with an ophthalmoscope, which allows doctors to look inside the eye.^1,2 Other data and information utilized in diagnosis-making, treatment, and management processes include personal and family health and medical history as well as immune system status.¹

People suspected of having papillitis undergo a variety of laboratory tests to pinpoint the possible origins of the infection and inflammation and trace its extent throughout the rest of the body.¹ Some procedures that would be relevant in making a diagnosis of papillitis include:

• Blood and serum testing
• Antibody testing in blood, serum, or cerebrospinal fluid (CSF)
• Tuberculin skin test and/or quantiferon blood test
• Computerized tomography (CT)
• Magnetic resonance imaging (MRI)¹

Treatment and management

Since papillitis is an infection, it can be treated and managed through the use of anti-infection and anti-inflammatory medications. These agents may be used to treat it on their own or in conjunction with corticosteroid therapy.¹ Depending on any conditions found to be connected to the development of papillitis, people affected also undergo disease-specific treatments and management regimens to address the signs and symptoms.

Prognosis

The potential treatment and management plans available to address papillitis and other conditions can vary greatly, depending on the goals and objectives of those diagnosed. However, while they may be able to address the infection and inflammation associated with papillitis, it varies significantly whether people with this diagnosis would recover their normal eye structures and eyesight.¹ To learn more about papillitis and its appearances in autoimmune and demyelinating disorders, continue reading below.

Autoimmune and demyelinating disorders related to papillitis

IgLON5 autoimmunity

IgLON5 Autoimmunity, also known as Anti-IgLON5 Disease, is a rare genetic condition in which the body produces antibodies that attack the protein IgLON5. This protein is created by the gene of the same name and typically works with nerve cells throughout the body to build and maintain their structures, functions, and connectivity with other nerve cells.^2,3 This condition occurs when the presence of this protein triggers the production of antibodies to attack them, convincing the body that the protein produced by the body is foreign. The autoimmune response results in the breakdown of these nerve cells and the development of various signs and symptoms.⁴

Signs and symptoms

While it is not typically found in cases of IgLON5 autoimmunity, the presence of papillitis has been reported in people with such a diagnosis. For some, it was the only physical manifestation of the autoimmune disorder, while others initially presented with a different set of signs and symptoms.² 

The other signs and symptoms typically seen in those with IgLON5 autoimmunity include:

• Sleeping problems
• Breathing difficulties, especially while sleeping
• Diminished cognitive abilities
• Irregular walking patterns and posture
• Irregular heartbeat
• Irregular breathing patterns³

Diagnosis

Diagnosing IgLON5 autoimmunity can be difficult because the signs and symptoms listed above are non-specific and may indicate the presence of different conditions. It's important to note that this disorder progresses slowly, so these and other signs and symptoms may not appear for some time.⁴ This can create obstacles in making an accurate diagnosis. Still, it is possible for healthcare professionals and people suspected of having this disorder to undergo diagnostic tests to eliminate differential diagnoses and reach this conclusion.

Since genetics plays an important role in the development processes of IgLON5 autoimmunity, genetic and molecular testing of the IgLON5 gene and the other related genes may yield the most accurate results.²

The wide range of signs and symptoms reported in people with this diagnosis will still be different in terms of how severe they are and when people first noticed them.³ Due to the genetic nature of this disorder, a thorough assessment is needed to get a clear understanding of how the disorder presents itself.

Other diagnostic tests that people suspected of having this disorder undergo to reach a definitive diagnosis of IgLON5 autoimmunity include:

• CSF test
• Blood immunohistochemistry
• Video polysomnography (VPSG)³

Treatment and management

Immunotherapy and anti-infection treatment form the current basis for treating and managing IgLON5 autoimmunity and papillitis, if found to be present and related to the autoimmune disorder. Intravenous corticosteroids, such as methylprednisolone, serve as the typical agents used in immunotherapy to treat and manage both disorders.^3,4 These medications are scheduled regularly to ensure that the body maintains adequate levels of these medications to avoid autoimmune responses. It is recommended to use more than one corticosteroid medication for the treatment and management regimens to be more effective at addressing the signs and symptoms of both the autoimmune disorder and the infection.³

Other medications that may be considered in treating and managing IgLON5 autoimmunity include: 

• Intravenous immunoglobulins
• Therapeutic Plasma Exchange (TPE)
• Rituximab
• Cyclophosphamide
• Azathioprine
• Mycophenolate³

Non-medication methods of IgLON5 autoimmunity treatment and management include:

• CPAP, BiLevel therapy, and other positive airway pressure treatments
• Intubation or tracheotomy^3,4

Autoimmune encephalitis

Encephalitis is an umbrella term for an infection and inflammation in the brain. Various infectious agents result in the development of this disorder, with the most commonly reported ones being viral and bacterial. Autoimmune encephalitis occurs when the body mistakenly produces antibodies that attack the brain tissue as a result of incorrectly identifying the tissue as foreign. ⁵

The presence of papillitis and other types of optic nerve disorders is not commonly reported in encephalitis, much less in those with an autoimmune cause. Glial Fibrillary Acidic Protein (GFAP) Antibody-Associated Encephalitis is one of the only types of Autoimmune Encephalitis that reports the presence of Papillitis.⁵

Signs and symptoms

Some signs and symptoms of the conditions that fall within the scope of autoimmune encephalitis include:

• Behavioural abnormalities, agitation, and other psychiatric manifestations
• Involuntary movements
• Seizures that are difficult to treat and manage
• Sleep disturbances
• Abnormal changes in heart rate, breathing, blood pressure, and temperature regulation
• Decreased level of consciousness
• Speaking and language understanding problems, memory problems, and other cognitive impairments⁶

Aside from papillitis, other signs and symptoms reported in GFAP Antibody-Associated Encephalitis include:

• Headache
• Encephalopathy
• Postural tremor⁵
• Short-term memory loss
• Presence of a tumour, especially in the ovaries
• Movement disorders⁶

Diagnosis

There’s a set of criteria typically used for diagnosing autoimmune encephalitis. Such a diagnosis is made when all three of the following categories are met:

• Short-term memory loss, altered mental status, or psychiatric signs and symptoms all develop and progress within less than three months
• At least one of the following is found:
  • Seizures not from a previously known seizure disorder
  • Increased white blood cell count in CSF
  • Possible encephalitis due to MRI features
  • New focal central nervous system (CNS) findings
• Exclusion of alternative causes⁶

The diagnostic and laboratory tests conducted to make an accurate diagnosis include:

• MRI
• Electroencephalogram (EEG)
• Blood, serum, and metabolic tests
• CSF analysis
• Tumour screening:
  • Serum and CSF analysis
  • PET/CT scan
  • Ultrasound or MRI⁶

Treatment and management

Currently, there is no standard for treating and managing autoimmune encephalitis. However, the outlook for those with this disorder is favourable if it was caught early enough and if treatment and management regimens begin quickly.⁶

The first round of treatment and management includes the following:

• Intravenous corticosteroids
• Intravenous immunoglobulin (IVIG)
• Plasma exchange⁶

In case the first round does not adequately treat and manage the signs and symptoms, the second round focuses on the use of Rituximab or Cyclophosphamide. For more severe cases of autoimmune encephalitis, agents such as Tocilizumab and Intravenous methotrexate is found to be effective in areas that are unresponsive to the first two.⁶

Maintenance therapies spanning years, depending on the person, are also recommended to control the signs and symptoms of this disorder and its progression. These regimens make recovery possible, even though it may take a while. The agents involved in these regimens include:

• IVIG
• Pulsed methylprednisolone
• Oral prednisone
• Azathioprine
• Mycophenolate⁶

Multiple sclerosis

Multiple Sclerosis (MS) is a chronic, progressive, and inflammatory autoimmune disorder that impacts the CNS of the body, which consists of the brain and spinal cord. This disorder develops when inflammatory lesions appear in nerve cells, causing deterioration of the myelin surrounding the axon. The loss of myelin in nerve cells disrupts the normal transmission of electrical signals between nerve cells, negatively impacting numerous bodily functions.⁷ 

Since it is a disorder that directly impacts the nervous system, it also presents signs and symptoms in all other body systems. For example, inflammation and infection cause the breakdown of the myelin tissue in the nerve cells of the optic nerve, leading to impaired normal eyesight and other optical functions. Optic neuritis, which includes papillitis, is one of the most commonly reported manifestations of this disorder.⁷

Signs and symptoms

Some of the other signs and symptoms reported by those with MS include:

• Muscle weakness in arms and legs
• Blurred vision due to Optic Neuritis
• Impaired sensory abilities
• Involuntary movements
• Diplopia⁷

Diagnosis

The diagnostic procedures involved in making a diagnosis of MS are also utilized in diagnosing papillitis, given their strong influence on the development of each other. These tests include:

• Comprehensive physical assessment
• Baseline chest x-ray
• MRI
• Blood, serum, and CSF tests
• CT
• Ultrasound
• Markers for autoimmune diseases
• Antibody tests⁸

Treatment and management

Until and long after a diagnosis of MS is made, which may take years due to its progressive nature, treatment and management mainly focus on addressing the signs and symptoms people present with. These plans also help treat and manage any adverse effects that arise as a result of MS, papillitis, or the treatment and management regimens of both. 

Intravenous corticosteroids are the first-line agents for treating and managing MS and papillitis. ⁷ Immunoglobulins are another group of medications known for their effectiveness in treating and managing MS. Methylprednisolone is an example of a corticosteroid medication known to be effective in addressing the signs and symptoms of papillitis and slowing the progression of MS. At regularly scheduled intervals, this agent helps minimize inflammation (characteristic of MS) with little to no side effects.⁸

Summary

• Papillitis is the infection and inflammation of the optic disc in the eye, which contains the optic nerve that allows for eyesight
• Papillitis can be either a standalone disorder or a manifestation of a related disease or condition
• Papillitis is typically reported alongside autoimmune and demyelinating disorders due to its inflammatory nature
• Papillitis is not present in all autoimmune and demyelinating disorders
• Some of the autoimmune and demyelinating disorders that report the presence of papillitis are IgLON5 autoimmunity, autoimmune encephalitis, and multiple sclerosis
• Intravenous corticosteroids are the front-line treatment and management agents for all three diseases and Papillitis, if it is reported