New Elmiron Study Suggests Established Maculopathy Patients Should be Asked About Possible Elmiron Use
Elmiron maculopathy secondary to pentosan polysulfate (PPS) toxicity was first reported in this 2018 Ophthalmology medical journal article, "Pigmentary Maculopathy Associated with Chronic Exposure to Pentosan Polysulfate Sodium". Now a new Elmiron medical study about cases of macular toxicity related to PPS exposure suggests that established maculopathy patients should be questioned regarding any Elmiron use in the past.
This new study was a retrospective case series of four patients with pigmentary maculopathy, and its findings are presented in this article, "New Insights Into Pentosan Polysulfate Maculopathy", posted on January 21, 2021, by the Ophthalmic Surgery, Lasers and Imaging Retina, or OSLI Retina.
From that January 2021 medical journal article about Elmiron maculopathy we get this background information set forth in its Introduction section:
Macular toxicity secondary to pentosan polysulfate sodium (PPS) (Elmiron; Janssen Pharmaceuticals, Beerse, Belgium) was first reported in 2018. PPS is the only U.S. Food and Drug Administration (FDA)-approved oral treatment for interstitial cystitis (IC), a painful urological condition2 affecting approximately 45 in 100,000 women and eight in 100,000 men globally. Pearce et al. initially described six patients with PPS exposure (approximately 400 mg/day, average of 15 years) presenting with pigmentary maculopathies characterized by paracentral hyperpigmentation and nodular excrescences on optical coherence tomography (OCT) at the level of the retinal pigment epithelium (RPE) with negative genetic testing.
Subsequent articles investigated the strength of the association, described the phenotypic range of presentation, and generated screening recommendations. Diagnostic criteria described include: 1) bilateral pathology centered on the fovea, 2) paracentral macular hyperpigmented spots, 3) dense array of hyper- and hypoautofluorescent spots and reticular fundus autofluorescence (FAF) abnormalities, and 4) foci of nodular RPE enlargement on OCT corresponding to hyperreflectance on near-infrared reflectance imaging. Humphrey visual fields (HVF) ranged from normal to dense paracentral to central scotomas correlating with the degree of atrophy and analysis of electroretinography (ERG) revealed normal to diffusely dampened amplitudes. [Footnotes omitted.]
Next, we look at the so-called "value-added" aspect of this new Elmiron medical study about cases of macular toxicity related to PPS exposure. From the Discussion section of the January 2021 OSLI Retina article:
This study provides novel information about the phenotype of PPS maculopathy. We demonstrate progression of FAF changes over 4 to 5 years (despite PPS discontinuation in one patient), collapse of nodular RPE excrescences over time, and quantification of drastic total retinal thinning in two patients during a 4- to 5-year follow-up period. We further showed FAZ abnormalities and choriocapillaris dropout on OCTA, and functional quantification of the previously reported subjective DA symptoms.
The strength of this analysis is the length of follow-up with incidental multimodal imaging over 4 years. FAF findings demonstrate increasing intensity and area of characteristic changes, and OCT data show progressive macular thinning. The choroidal thickness during the same period remained stable, whether that implies a degree of direct retinal toxicity remains to be elucidated....
Another contribution of this new Elmiron medical study to the medical literature has to do, in large part, with how one might diagnose Elmiron maculopathy secondary to pentosan polysulfate (PPS) toxicity. From the Conclusions section of the January 2021 OSLI Retina article:
Patients with long-term [use of Elmiron, i.e., exposure to PPS] may present with various pre-existing diagnoses but most PPS toxicity cases manifest with a specific constellation of OCT, FAF, and [OCT angiography (OCTA)] findings. Established maculopathy patients should be questioned regarding exposure to PPS, especially with negative genetic testing or atypical presentations. For patients with [cystoid macular edema (CME)], [carbonic anhydrase inhibitors (CAIs)] can be effective and may need to be continued long after the [Elmiron (PPS)] has been withdrawn. Patients can have progressive RPE changes even after [Elmiron] medication discontinuation, and vascular changes may imply mechanisms of toxicity involving both the choriocapillaris and RPE. The finding of significant choriocapillaris dropout may inform understanding of the pathophysiology.
Our law firm is investigating possible Elmiron lawsuits for patients who have suffered vision loss due to Elmiron maculopathy. If we can be of assistance to you or someone else regarding an Elmiron vision loss case, you can send an email to Tom Lamb or you can call us on our toll-free number: 800-426-9535.
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