Dr Jasmine H. Francis and colleagues have reported a case of Refractory Nodular Scleritis that has been published in the journal of American Medical Association.
Scleritis is a chronic, painful, and potentially blinding inflammatory disease.It is characterized by edema and cellular infiltration of the scleral and episcleral tissues. It has an underlying cause, usually an autoimmune disease including rheumatoid arthritis, granulomatosis with polyangiitis, other vasculitic/connective tissue diseases.
According to history, a 59-year-old woman with a 5-year history of intermittent
bilateral uveitis and scleritis presented with painful left uveitis and an
episcleral nodule. Ultrasound biomicroscopy revealed an elevated and tender
uveal-episcleral lesion. Visual acuity was 20/30 in the left eye and 20/25 in
the right eye. She had been taking 60 mg of oral prednisone; topical
prednisolone acetate, 1%; and oral ibuprofen for 10 days without relief. She
was suspected of being a steroid responder due to elevated intraocular pressure
bilaterally. Three years prior, sinus pressure symptoms led to magnetic
resonance imaging that revealed a nasal sinus mass. Biopsy demonstrated a
histiocytic infiltrate with emperipolesis, consistent with
Rosai-Dorfman-Destombes (RDD) disease. Timolol-dorzolamide topical drops were
initiated. Staging evaluations showed no other sites of RDD disease.
The patient was diagnosed having Episcleral-uveal nodule and
uveitis associated with histiocytosis. Next line was treating the left eye with
Uveitis and episcleral/uveal tract nodules can be
manifestations of RDD disease, but RDD disease can be refractory to steroids,
making an alternative treatment necessary. RDD disease is a subtype of
histiocytosis, in which clonal expansion of activated histiocytes accumulate in
tissues including the lymph nodes and possibly the eye. Given the myelogenous
derivation of histiocytic disease, targeted delivery of melphalan is emerging
as a treatment option for isolated, symptomatic disease, particularly of ocular
structures. It is standard care for retinoblastoma and has few adverse events.
Following 3 monthly infusions of intra-arterial melphalan (9 mg) administered
to the ophthalmic artery, the episcleral/uveal nodule regressed, the pain
abated, oral steroids and all topical drops were discontinued, and the
corrected visual acuity improved to 20/20.
Histiocytoses are rare hematologic disorders characterized
by pathological accumulation and infiltration of activated histiocytes in
affected tissues, including ophthalmic structures. The non– Langerhans cell
histiocytoses include RDD disease and are clinically heterogenous, including
vision-threatening structures (such as the orbit, uveal tract, optic nerves, or
the hypothalamic-pituitary adrenal axis). This case demonstrated an episcleral
nodule with sclerouveal infiltration seen with ultrasound biomicroscopy.
Despite oral and topical steroids, she had no improvement.
Because histiocytic neoplasms are hematologic in their
pathogenesis, authors considered intra-arterial melphalan as a potential
therapeutic option. Melphalan is an alkylating agent of mustard gas derivation
used for blood-based neoplasms, notably multiple myeloma. The advantage of
intra-arterial chemotherapy is delivering a therapeutic dose to the target
lesion while minimizing systemic administration.
This patient had no myelosuppression or other systemic toxic
reactions following her 3 courses of 9-mg intra-arterial melphalan. Instead,
she had a sustained ocular response, which allowed her to discontinue systemic
steroids, oral nonsteroidal anti-inflammatory drugs, and all eye drops. She
continued to have a sustained response at 10 months’ follow-up.
In summary, authors highlighted that ocular histiocytosis
may masquerade as refractory nodular scleritis. They describe implementation of
intra-arterial chemotherapy for this vision-threatening intraocular
histiocytosis with sclerouveal infiltration. This treatment approach might
benefit from broader evaluation of its feasibility and safety across a variety
of tumor sites and arterial targets, as well as its efficacy.
H. Francis, MD; Y. Pierre Gobin, MD; Eli L. Diamond, MD; JAMA Ophthalmology