Phacoanaphylactic Endophthalmitis

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Disease Entity

ICD 10 code: H44.19

Endophthalmitis: other


Disease

Lens-induced uveitis (LIU) is an uncommon autoinflammatory condition where the immune system’s natural tolerance of lens protein is abandoned, most often occurring after traumatic or surgical lens injury. This disease is also known by other names including phacoanaphylactic endophthalmitis, phacogenic uveitis, and phacotoxic uveitis 1. It was first described in medical literature in 1919 and termed “endophthalmitis anaphylactica” in a 1922 case series of patients who developed non-infectious endophthalmitis days to weeks following extracapsular cataract surgery 2. Contrary to what is suggested by such a name, this disease does not involve true anaphylaxis and does not feature an IgE-mediated response. Lens-induced uveitis remains a small fraction of clinically encountered cases of uveitis, especially with modern surgical techniques that include complete removal of the lens during cataract removal 3.

Etiology

Lens-induced uveitis is thought to be the result of an abnormal immune tolerance to lens proteins, resulting in a cell-mediated autoimmune phenomenon. Researchers have argued against the mechanism of immune privilege and rejection of newly encountered self antigens, noting the rare frequency of this condition despite the presence of extracapsular lens protein in asymptomatic individuals. Instead, this condition is proposed to be caused by an abnormal recognition of self antigens on lens material 1.

Risk Factors

Violation of the lens capsule during trauma or surgery is a known risk factor for lens-induced uveitis. Patients with hypermature cataracts are also at an increased risk of developing this condition as degrading lens material is known to leak through an intact capsule, triggering an autoinflammatory response. The primary affected demographic is 50-79 years of age, however younger patients can also be affected.

General Pathology

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Pathophysiology

The pathophysiology of this disease begins with introduction of the lens protein to the immune system. While this can be the result of traumatic or surgical injury to the lens or surrounding lens capsule, these proteins may also come into contact with the extracapsular space due to leakage through the capsule in advanced cataracts in the absence of trauma to the lens. In post-operative cases, retained cortical fragments may also trigger an autoinflammatory state.

Despite the name phacoanaphylactic endophthalmitis, this disease is not a IgE mediated response . Rather, it involves an immune complex-mediated mechanism that is proposed to follow the loss of T-cell tolerance of lens proteins. This will classically result in a granulomatous reaction mediated by monocytes and polymorphonuclear cells to lens protein. This process can involve a type II, III, or type IV hypersensitivity reaction 1. Clinical symptoms typically emerge between days to months following the inciting event and can range from mild to very severe endophthalmitis 2.

Primary prevention

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Diagnosis

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History

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Physical examination

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Signs

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Symptoms

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Clinical diagnosis

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Diagnostic procedures

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Laboratory test

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Differential diagnosis

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Management

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General treatment

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Medical therapy

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Medical follow up

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Surgery

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Surgical follow up

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Complications

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Prognosis

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Additional Resources

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References

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