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PubMed This is a summary of 17 peer-reviewed journal articles Updated
Ophthalmology

Your Care Team and Long-term Monitoring

At a Glance

Scleromalacia perforans can continue to thin the eye wall or cause complications even when the eye looks quiet. Long-term follow-up with an eye specialist and rheumatologist helps monitor vision, eye pressure, eye-wall thickness, medication effects, and warning signs in other organs.

Managing scleromalacia perforans (SP) requires careful coordination. Because the eye wall can continue to thin even after the “active” phase of the disease has passed, you need a specialized team that monitors not just your sight, but your entire body [1][2].

Your Core Care Team

Because SP is a complex cross-disciplinary condition, your care should be managed by a team of experts:

  • Ophthalmology Specialists: You should ideally be seen by a uveitis specialist (an expert in ocular inflammation) or a cornea/anterior segment specialist. These doctors use serial examinations and tools, like Anterior-Segment OCT, to measure subtle changes in your scleral thickness [3][4].
  • Rheumatologist: This doctor is the “quarterback” for your systemic health. They manage the immunosuppressive medications that address the inflammation and monitor you for signs that the disease is affecting other organs [5][6].
  • Internal Medicine/PCP: Your primary doctor ensures that your general health—such as bone density and infection risk—is maintained while you are on powerful medications.

Long-Term Eye Complications

Even if your eye looks “quiet,” the structural changes caused by SP can lead to secondary issues over time. Monitoring is essential to catch these early:

  • Secondary Glaucoma: The inflammation or the steroids used to treat it can cause the pressure inside your eye to rise, which can damage the optic nerve [7][8].
  • Cataracts: Clouding of the eye’s natural lens is a known risk for patients with necrotizing scleritis and those taking systemic corticosteroids [9][10]. The specific risk depends on the underlying inflammation, prior surgery, and steroid exposure.
  • Hypotony (Low Pressure): If the eye wall becomes too thin or if the internal structures that produce eye fluid are damaged, the eye pressure can drop too low, which can also affect vision [11][12].
  • Vision Loss: In severe cases, structural failure can lead to retinal detachment or the need for a surgical patch graft to save the globe [8][9].

The Importance of Systemic Monitoring

One of the most critical reasons for long-term care is the risk of “isolated” scleritis turning into a systemic disease.

  • The Risk of Systemic Disease: Research in a particular clinical cohort has shown that among patients who initially only have eye symptoms but test positive for ANCA antibodies, a significant percentage will develop systemic ANCA-associated vasculitis (AAV) during long-term follow-up [13].
  • Warning Signs Beyond the Eye: Your rheumatologist will watch for “red flags” that the disease is moving to your lungs or kidneys. These include persistent sinus pain, nosebleeds (epistaxis), a chronic cough, or blood in your urine [14][15].
  • Inflammatory Markers: If your C-Reactive Protein (CRP) was high when you were first diagnosed, you may be at a higher risk for the disease becoming systemic, requiring more frequent check-ins [13].

A Lifetime of Vigilance

Even if your eye has been stable for years, recurrences can happen, sometimes at a different spot on the eye or after you begin to taper off your medications [8][16]. A “stable” eye in scleromalacia perforans is one that is being watched closely by a team that talks to each other. Clinical examination, visual acuity, intraocular pressure, and serial photographs are central tools for ensuring that “quiet” doesn’t mean “unnoticed” [17][3].

Common questions in this guide

Which doctors should be involved in my care for scleromalacia perforans?
Care commonly includes an ophthalmologist with expertise in uveitis or cornea and anterior-segment disease, along with a rheumatologist. A primary care doctor can help monitor general health, infection risk, and medication effects and keep the team connected.
How is a quiet eye monitored in scleromalacia perforans?
Even when symptoms or visible inflammation are quiet, follow-up may include vision testing, eye-pressure checks, detailed examinations, photographs, and measurements of eye-wall thickness with anterior-segment OCT. Your ophthalmologist will choose the timing based on your eye findings, blood tests, medicines, and history.
What complications can develop over time?
Long-term thinning or inflammation, and some treatments, can contribute to glaucoma, cataracts, or abnormally low eye pressure. Severe structural damage can threaten vision and may lead to retinal detachment or the need for a patch graft.
What symptoms could mean scleromalacia perforans is affecting other organs?
New persistent sinus pain, nosebleeds, a chronic cough, or blood or unusual color in the urine can be warning signs of inflammation involving the nose, lungs, or kidneys. Tell your rheumatologist or other clinician about these symptoms promptly, especially if ANCA antibodies are positive.
Do ANCA or CRP results affect follow-up?
Positive ANCA antibodies and a high CRP at diagnosis may be linked with a greater risk that eye-limited disease will later become systemic ANCA-associated vasculitis. Your rheumatologist may use these results, along with symptoms and examinations, to decide how closely to screen your kidneys and lungs.
Can scleromalacia perforans recur after years of stability?
Yes. Scleromalacia perforans can recur after a long stable period, including during or after medication tapering, and it may appear in a different area of the eye. Continued scheduled monitoring is important even when you have little or no pain.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.How many patients with necrotizing scleritis or scleromalacia perforans have you managed, and how do you coordinate care with a rheumatologist?
  2. 2.Given that my eye is currently 'quiet,' how often do we need to check my scleral thickness to ensure the thinning isn't slowly progressing?
  3. 3.What is my current intraocular pressure, and are there signs that I am developing secondary glaucoma or cataracts?
  4. 4.If my ANCA or CRP tests were positive, what is our long-term plan for screening my kidneys and lungs for systemic vasculitis?
  5. 5.Are you and my rheumatologist in direct communication regarding my blood test results and any potential side effects from my immunosuppressants?
  6. 6.At what point would you recommend a referral to a uveitis or cornea specialist for more intensive monitoring?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (17)
  1. 1

    Dengue fever-associated necrotizing scleritis: A case report with long-term follow-up.

    Kamoi K, Mochizuki M, Ohno-Matsui K

    Medicine 2018; (97(32)):e11875 doi:10.1097/MD.0000000000011875.

    PMID: 30095666
  2. 2

    Sclerokeratitis and Secondary Glaucoma in Relapsing Polychondritis in a 30-Year-Old Asian Male Patient: A Case Report.

    Flores LKC, Siazon RR

    Cureus 2025; (17(2)):e79128 doi:10.7759/cureus.79128.

    PMID: 40109806
  3. 3

    Anterior segment spectral domain optical coherence tomography imaging of patients with anterior scleritis.

    Levison AL, Lowder CY, Baynes KM, et al.

    International ophthalmology 2016; (36(4)):499-508 doi:10.1007/s10792-015-0153-8.

    PMID: 26597942
  4. 4

    Case Report: Subconjunctival Triamcinolone Acetate-associated Scleral Necrosis: Is It Really Obsolete?

    Agarwal R, Bafna RK, Chetan C, Sharma N

    Optometry and vision science : official publication of the American Academy of Optometry 2021; (98(9)):1016-1020 doi:10.1097/OPX.0000000000001761.

    PMID: 34433205
  5. 5

    [Diagnostic and Therapeutic Management of Episcleritis and Scleritis].

    Turgut F, Dingerkus V, Tappeiner C, Becker M

    Klinische Monatsblatter fur Augenheilkunde 2023; (240(5)):725-738 doi:10.1055/a-2022-0689.

    PMID: 36827997
  6. 6

    Necrotizing Scleritis: A Review.

    Dutta Majumder P, Agarwal S, Shah M, et al.

    Ocular immunology and inflammation 2024; (32(7)):1405-1419 doi:10.1080/09273948.2023.2206898.

    PMID: 37279404
  7. 7

    Scleritis associated with relapsing polychondritis.

    Sainz-de-la-Maza M, Molina N, Gonzalez-Gonzalez LA, et al.

    The British journal of ophthalmology 2016; (100(9)):1290-4 doi:10.1136/bjophthalmol-2015-306902.

    PMID: 26888976
  8. 8

    Ocular complications and mortality in peripheral ulcerative keratitis and necrotising scleritis: The role of systemic immunosuppression.

    Ogra S, Sims JL, McGhee CNJ, Niederer RL

    Clinical & experimental ophthalmology 2020; (48(4)):434-441 doi:10.1111/ceo.13709.

    PMID: 31872475
  9. 9

    Clinical Profile of Patients with Necrotizing Scleritis in a Tertiary Eye Care Center in Southern India.

    Dutta Majumder P, Ghose A, Chidambaram M, et al.

    Ocular immunology and inflammation 2018; (26(3)):412-416 doi:10.1080/09273948.2016.1223857.

    PMID: 27762661
  10. 10

    Surgically induced necrotizing scleritis (SINS): Is it a standalone condition or a variant of necrotizing scleritis?

    Magesan K, Dutta Majumder P, Agarwal M, et al.

    Indian journal of ophthalmology 2024; (72(Suppl 4)):S592-S595 doi:10.4103/IJO.IJO_2724_23.

    PMID: 38622865
  11. 11

    Infective Necrotizing Scleritis After XEN Gel Stent With Mitomycin-C.

    Kingston EJ, Zagora SL, Symes RJ, et al.

    Journal of glaucoma 2022; (31(2)):129-132 doi:10.1097/IJG.0000000000001959.

    PMID: 34731869
  12. 12

    Choroidal involvement in non-infectious posterior scleritis.

    Vermeirsch S, Testi I, Pavesio C

    Journal of ophthalmic inflammation and infection 2021; (11(1)):41 doi:10.1186/s12348-021-00269-9.

    PMID: 34705127
  13. 13

    ANCA-associated scleritis: impact of ANCA on presentation, response to therapy and outcome.

    Perray L, Nguyen Y, Clavel Refregiers G, et al.

    Rheumatology (Oxford, England) 2024; (63(2)):329-337 doi:10.1093/rheumatology/kead252.

    PMID: 37233203
  14. 14

    Clinical characteristics of patients with granulomatosis with polyangiitis and microscopic polyangiitis in ENT practice: a comparative analysis.

    Wojciechowska J, KręCicki T

    Acta otorhinolaryngologica Italica : organo ufficiale della Societa italiana di otorinolaringologia e chirurgia cervico-facciale 2018; (38(6)):517-527 doi:10.14639/0392-100X-1776.

    PMID: 30623897
  15. 15

    A Rare Case of Nephrotic-Range Proteinuria in Antineutrophil Cytoplasmic Antibodies (ANCA)-Associated Vasculitis.

    Nasr R, Balasubramanian P, Desiderio L, Abdelattif M

    Cureus 2022; (14(5)):e24889 doi:10.7759/cureus.24889.

    PMID: 35572457
  16. 16

    Multifocal Surgically Induced Necrotizing Scleritis Following Strabismus Surgery: A Case Report.

    Akbari MR, Mohebbi M, Johari M, et al.

    Strabismus 2016; (24(3)):101-5 doi:10.1080/09273972.2016.1205104.

    PMID: 27485819
  17. 17

    Monitoring therapy in anterior necrotizing scleritis with inflammation with anterior segment optical coherence tomography.

    Vidal Oliver L, López Montero A, Gil Hernández I, et al.

    European journal of ophthalmology 2021; 11206721211065564 doi:10.1177/11206721211065564.

    PMID: 34866457

This information about care-team coordination and long-term monitoring for scleromalacia perforans is for informational purposes only and does not constitute medical advice. Your ophthalmologist and rheumatologist should tailor follow-up and testing to your situation.

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