MAR 01, 2020
Macular Findings Point to Immunoproliferative Disorder
By Jean Shaw
Selected By: Andrew P. Schachat, MD
Retina/Vitreous
Journal Highlights
Ophthalmology Retina, March 2020
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Smith et al. evaluated four patients with neurosensory macular detachment and asymptomatic monoclonal gammopathy of undetermined significance (MGUS). They found that angiographically silent and treatment-resistant submacular fluid may point to an underlying immunoproliferative subset of MGUS, which they term monoclonal gammopathy of macular significance. In addition, they found that the condition developed into malignant disease in three of the four patients. Given this heightened risk of malignancy, they recommend that patients with neurosensory macular detachment not attributable to known causes should undergo serum protein analysis.
For this retrospective case series, the researchers gathered clinical, laboratory, and imaging findings for four patients with refractory serous macular detachment associated with MGUS. Two of the patients were diagnosed incidentally with MGUS by their primary care team while being followed up for chronic submacular fluid; the others were diagnosed after their retina specialist ordered specific laboratory tests for immunogammopathy.
Of eight eyes, seven demonstrated neurosensory macular detachment with treatment-resistant submacular fluid and vitelliform material. None of the involved eyes showed signs of significant hyperviscosity retinopathy, and no definite leakage was evident on fluorescein angiography. All four patients were resistant to treatments aimed at resolving the subretinal fluid, including anti-VEGF drugs, photodynamic therapy, and topical dorzolamide.
Three of the four patients underwent malignant transformation (two to multiple myeloma, and one to lymphoplasmacytic lymphoma). The fourth patient remained free of any malignancy eight years after diagnosis.
This case series illustrates that MGUS may rarely be associated with sub- and intraretinal fluid that is angiographically silent. The authors hypothesize that preexisting chorioretinal disease—and, in some patients, genetic factors—may predispose patients with MGUS to macular fluid accumulation, and they recommend lab evaluation for immunogammopathy in patients who have serous macular detachment that cannot be attributed to known causes.
The original article can be found here.