Scleromalacia perforans, or non-inflammatory necrotizing scleritis, is a severe and very rare form of scleritis.
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Epidemiology
Associations
Clinical presentation
The condition is generally asymptomatic. It is usually found on visual inspection during a formal eye examination.
Pathology
Scleromalacia perforans is a rare non-inflammatory form of anterior necrotizing scleritis, usually seen in patients with autoimmune disease.
Radiographic features
The scleral thinning may secondarily calcify, and can be seen on CT. In addition, the rare sequela of atraumatic globe rupture might be present. Other manifestations of the underlying autoimmune disease may also be present.
Treatment and prognosis
The condition is usually asymptomatic. Rupture of the globe is rare and usually only seen in those with coexisting markedly elevated intraocular pressure, i.e. severe glaucoma 1.
Historically the condition has shown a limited response to conventional treatments. Treatment options include 6:
non-steroidal anti-inflammatory drugs (NSAIDs)
topical treatments: glucocorticoids, tacrolimus, cyclosporin
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systemic immunosuppressants:
glucocorticoids
TNF-alpha inhibitors
other immunosuppressants: cyclophosphamide, mycophenolate, azathioprine, methotrexate, rituximab, tocilizumab, tofacitinib
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