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Frontiers in Nephrology 2024A 27-year-old female at 20th week of pregnancy was admitted with edema, foamy urine, but normal blood pressure. Her blood count was normal, she had proteinuria of 3...
A 27-year-old female at 20th week of pregnancy was admitted with edema, foamy urine, but normal blood pressure. Her blood count was normal, she had proteinuria of 3 g/day, creatinine 0.4 mg/dl, albumin 2.4 g/dl, and cholesterol 355 mg/dl. Antinuclear antibodies 1/160, but Anti-DNA, anticardiolipin antibodies and lupus anticoagulant were negative, with normal serum C3 and C4. A renal biopsy showed secondary membranous glomerulopathy, most likely lupus class V pure. Steroids, azathioprine, and aspirin were initiated, up to 28 weeks of pregnancy, when she developed severe hypertension, photopsia, headache, anasarca, extensive bruising of the extremities, severe anemia, thrombocytopenia, and creatinine rose to 2.09 mg/dl with preserved diuresis. A female infant, 1045 grams, was delivered by emergency caesarean section. Following the surgery, she experienced diplopia, dysarthria, bradypsychia, and sensory alterations in the lower extremities, necessitating emergency hemodialysis due to pulmonary congestion. Blood smear revealed schistocytes, LDH elevated at 1148 IU/L, while transaminases and liver function remained normal, suggesting thrombotic thrombocytopenic purpura. ADAMTS13 revealed 6% activity with the presence of inhibitor. Mycophenolate and daily plasmapheresis with fresh frozen plasma replacement yielded unsatisfactory response, unaffected by the addition of methylprednisolone pulses and rituximab. Eventually, intravenous cyclophosphamide was introduced, resulting in complete hematological remission and normalization of ADAMTS13, however dialysis-dependence persisted and four years later, right renal cancer prompted bilateral nephrectomy. After a total follow-up of six years, she remained free of neoplastic recurrence and lupus activity, receiving prednisone and hydroxychloroquine. The differential diagnosis of microangiopathic syndrome in a pregnant lupus patient is discussed.
PubMed: 38379641
DOI: 10.3389/fneph.2024.1343594 -
European Journal of Case Reports in... 2024Most lung cancers are diagnosed at an advanced stage. Common metastatic sites include the brain, bone, liver and adrenal glands. Ocular metastases, however, are...
INTRODUCTION
Most lung cancers are diagnosed at an advanced stage. Common metastatic sites include the brain, bone, liver and adrenal glands. Ocular metastases, however, are extremely rare. We present a case of advanced lung adenocarcinoma presenting exclusively with photopsias attributable to retinal metastases.
CASE DESCRIPTION
We describe a woman in her fifties, a lifetime non-smoker with an unremarkable medical and family history, who presented to the emergency department with photopsias for a week. Ophthalmology evaluation revealed decreased visual acuity bilaterally, and a fundus examination disclosed lesions suggestive of bilateral retinal metastases. A comprehensive evaluation diagnosed a stage IVb lung adenocarcinoma with exon 19 mutation on epidermal growth factor receptor gene. Subsequently, she developed complaints of headaches and dizziness. She received frontline osimertinib 80 mg daily, preceded by upfront whole-brain radiation therapy with partial orbital inclusion for symptomatic ocular and brain metastases. After ten radiation therapy sessions, her complaints were resolved and an ophthalmology revaluation revealed improvement in visual acuity and resolution of photopsia complaints. The patient is currently on osimertinib and preserves an ECOG score of 0.
CONCLUSION
Retinal metastases usually indicate advanced disease, so presenting with isolated ocular symptoms is exceedingly rare. Especially in cases of uncommon metastases, a multidisciplinary approach is fundamental for a prompt diagnosis and timely treatment, impacting prognosis and quality of life.
LEARNING POINTS
Ocular metastases in lung cancer are usually a sign of advanced disease.Advanced lung adenocarcinoma presenting solely with retinal metastases is extremely rare.A multidisciplinary team is essential for the diagnosis and treatment of lung cancer with uncommon metastases.
PubMed: 38223273
DOI: 10.12890/2023_004190 -
BMC Ophthalmology Jan 2024Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare presumed inflammatory chorioretinopathy characterized by creamy, yellow-white placoid...
BACKGROUND
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare presumed inflammatory chorioretinopathy characterized by creamy, yellow-white placoid lesions at the level of the retinal pigment epithelium (RPE). Unilateral cases often have fellow eye involvement within days to a few weeks. This report details a rare case of delayed contralateral APMPPE, in which unilateral lesion resolution was followed by contralateral eye involvement 31 months later.
CASE PRESENTATION
A 38-year-old woman presented with three days of blurry vision and photopsias in the right eye (OD). She endorsed a viral GI illness one month prior. Visual acuity was 20/25 -2 OD and 20/20 -1 in the left eye (OS). Examination revealed creamy, yellow-white placoid lesions in the posterior pole. Fluorescein angiography (FA) was notable for early hypofluorescence and late hyperfluorescence of the lesions, consistent with APMPPE. MRI and MRA brain were negative for cerebral vasculitis. She was treated with oral prednisone with complete resolution of her symptoms, vision, and lesion regression. She then presented 31 months later, with blurry vision OS and similar new creamy, yellow-white placoid lesions in the posterior pole OS. She endorsed receiving an influenza vaccine one month prior. FA again was notable for early hypofluorescence. She was diagnosed with APMPPE, this time involving the left eye, and was once again started on oral steroids with complete resolution. She denied any neurologic symptoms.
CONCLUSIONS
APMPPE is an inflammatory vasculitis of the choroid, leading to hypoperfusion and ischemic injury of the RPE with subsequent lesion formation. APMPPE may be preceded by a viral prodrome or vaccination, both of which were seen in this case. Choroidal inflammation seen in APMPPE is therefore thought to stem from immune-mediated processes. Unilateral cases often have fellow eye involvement within days to a few weeks. Single eye involvement with delayed contralateral presentation, as seen in our patient, is rare. This case demonstrates that lesion resolution in one eye can be followed by contralateral eye involvement up to 31 months later, highlighting the importance of routine ophthalmic monitoring for patients with unilateral APMPPE.
Topics: Humans; Female; Adult; White Dot Syndromes; Choroid; Inflammation; Retinal Pigment Epithelium; Face
PubMed: 38195467
DOI: 10.1186/s12886-023-03221-8 -
ESC Heart Failure Apr 2024A therapeutic strategy for chronic heart failure (HF) is to lower resting heart rate (HR). Ivabradine is a well-known HR-lowering agent, but limited prospective data... (Observational Study)
Observational Study
AIMS
A therapeutic strategy for chronic heart failure (HF) is to lower resting heart rate (HR). Ivabradine is a well-known HR-lowering agent, but limited prospective data exist regarding its use in Chinese patients. This study aimed to evaluate the effectiveness and safety of ivabradine in Chinese patients with chronic HF.
METHODS AND RESULTS
This multicentre, single-arm, prospective, observational study enrolled Chinese patients with chronic HF. The primary outcome was change from baseline in HR at 1 and 6 months, measured by pulse counting. Effectiveness was also evaluated using laboratory tests, the Kansas City Cardiomyopathy Questionnaire (KCCQ) clinical summary score (CSS) and overall summary score (OSS), and New York Heart Association (NYHA) class. Treatment-emergent adverse events (TEAEs) were assessed. A post hoc analysis examined the effectiveness and safety of ivabradine combined with an angiotensin receptor-neprilysin inhibitor (ARNI) or beta-blocker. A total of 1003 patients were enrolled [mean age 54.4 ± 15.0 years, 773 male (77.1%), mean baseline HR 88.5 ± 11.3 b.p.m., mean blood pressure 115.7/74.4 ± 17.2/12.3 mmHg, mean left ventricular ejection fraction 30.9 ± 7.6%, NYHA Classes III and IV in 48.8% and 22.0% of patients, respectively]. HR decreased by a mean of 12.9 and 16.1 b.p.m. after 1 and 6 months, respectively (both P < 0.001). At Month 6, improvements in the KCCQ CSS and OSS of ≥5 points were observed in 72.1% and 74.1% of patients, respectively (both P < 0.001). Left ventricular ejection fraction increased by 12.1 ± 11.6 (P < 0.001), and 66.7% of patients showed improvement in NYHA class (P < 0.001). At Month 6, the overall proportion of patients in NYHA Classes III and IV was reduced to 13.5% and 2.1%, respectively. Serum brain natriuretic peptide (BNP) and N-terminal pro-BNP changed by -331.9 ng/L (-1238.6, -134.0) and -1113.8 ng/L (-2202.0, -297.2), respectively (P < 0.001). HR reductions and improvements in NYHA and KCCQ scores with ivabradine were similar with and without use of ARNIs or beta-blockers. Of 498 TEAEs in 296 patients (29.5%), 73 TEAEs in 55 patients (5.5%) were considered related to ivabradine [most frequent sinus bradycardia (n = 7) and photopsia (n = 7)]. TEAEs were reported in a similar number of patients in ARNI and beta-blocker subgroups (21.9-35.6%).
CONCLUSIONS
Ivabradine treatment reduced HR and improved cardiac function and health-related quality of life in Chinese patients with chronic HF. Benefits were seen irrespective of whether or not patients were also taking ARNIs or beta-blockers. Treatment was well tolerated with a similar profile to previous ivabradine studies.
Topics: Adult; Aged; Humans; Male; Middle Aged; Adrenergic beta-Antagonists; Benzazepines; Cardiovascular Agents; China; Heart Failure; Ivabradine; Prospective Studies; Quality of Life; Stroke Volume; Treatment Outcome; Ventricular Function, Left; Vision Disorders; Female
PubMed: 38193606
DOI: 10.1002/ehf2.14581 -
Current Hypertension Reports Apr 2024This review summarizes key findings relating to the association between preeclampsia and retinal disorders. (Review)
Review
PURPOSE OF REVIEW
This review summarizes key findings relating to the association between preeclampsia and retinal disorders.
RECENT FINDINGS
Preeclampsia is a major cause of maternal morbidity. Pregnant women with preeclampsia frequently describe having visual disturbances. Retinal changes can be identified on fundoscopy in most patients with preeclampsia. While retinal pathology secondary to preeclampsia usually resolves postpartum, there is growing evidence that women with preeclampsia have a higher long-term risk of developing retinal disorders after pregnancy. Pregnant women often experience visual changes. While these symptoms may be benign, careful attention should be paid to exclude retinal disorders secondary to preeclampsia. Pregnant women complaining of new-onset or worsening blurry vision, scotomata, diplopia, or photopsia require rapid and thorough evaluation to rule out hypertensive disorders. Management of preeclampsia, including administration of magnesium sulfate and delivery of the fetus, can reverse retinal pathologies in most cases.
Topics: Female; Humans; Pregnancy; Pre-Eclampsia; Hypertension; Vision Disorders; Retina
PubMed: 38133842
DOI: 10.1007/s11906-023-01290-0 -
BMC Ophthalmology Oct 2023As the trend of refractive lens exchange for presbyopia continues to grow, our case report shows the first occurrence of an acute bilateral outer retinopathy following...
BACKGROUND
As the trend of refractive lens exchange for presbyopia continues to grow, our case report shows the first occurrence of an acute bilateral outer retinopathy following uncomplicated sequential clear lens extraction in an otherwise healthy individual.
CASE PRESENTATION
A 54-year-old male without significant medical history benefited from a sequential bilateral lens exchange for presbyopia. He then experienced a rapid vision loss in both eyes, accompanied by photopsias and myodesopsias, with symptoms appearing respectively 4 and 3 weeks after the surgeries. Multimodal imaging revealed a fulminant outer retinopathy, leading to a total loss of light perception within a few days. Immediate intravenous corticosteroid therapy was administered, permitting to recover a small area of central visual function in both eyes, enabling shape and color distinction. The primary diagnostic hypothesis is a presumed autoimmune retinopathy, triggered by the cataract extraction, while an alternative diagnosis could be a toxic reaction secondary to the use of intracameral cefuroxime and lidocaine during the surgery.
CONCLUSION
In this report, the authors describe the first recorded instance of outer retinopathy following cataract surgery. This occurrence raises the possibility of auto-immunization leading to retinal atrophy and vision loss as a potential outcome after undergoing cataract surgery.
Topics: Male; Humans; Middle Aged; Presbyopia; Autoimmune Diseases; Cataract Extraction; Blindness; Vision Disorders; Retinal Degeneration; Cataract
PubMed: 37872478
DOI: 10.1186/s12886-023-03171-1 -
Retinal Cases & Brief Reports Sep 2023To describe idiopathic multifocal choroiditis (iMFC) with chorioretinal atrophy developing choroidal flow voids on optical coherence tomography angiography (OCTA) that...
PURPOSE
To describe idiopathic multifocal choroiditis (iMFC) with chorioretinal atrophy developing choroidal flow voids on optical coherence tomography angiography (OCTA) that preceded a recurrence of the disease.
METHODS
Case report.
RESULTS
A 24-year-old woman presented with visual field changes and occasional photopsias. Systemic work-up for syphilis, tuberculosis, and sarcoidosis, was negative. Clinical findings and multimodal imaging were consistent with iMFC with chorioretinal atrophy, complicated by inactive choroidal neovascularization in her right eye. She was treated with systemic corticosteroids with a taper over 3 months without change in her examination. She was then stable for two years. At that point, the patient experienced increased photopsias, but her examination was unchanged. OCTA showed multiple flow voids in the choroid that were not present 6 months prior. No lesions were seen on other imaging modalities. Structural OCT showed some subtle hyper-reflectivity throughout the choroid that was previously absent. Given the unknown significance of these flow voids, the patient was asked to return for follow-up in one month. Her photopsias improved and her vision remained normal. On repeat examination after one month, the patient had developed a few subtle yellow lesions in the supero-nasal quadrant of the left eye. There were no macular lesions. The repeat OCTA revealed that the flow voids were fading.
CONCLUSION
Imaging findings using OCTA in our patient with iMFC showed choroidal flow voids that preceded clinical recurrence, not detected by other imaging modalities. Future studies should determine if OCTA can be used to detect subclinical lesions preceding clinical recurrences of iMFC.
PubMed: 37769259
DOI: 10.1097/ICB.0000000000001501 -
Retina (Philadelphia, Pa.) Nov 2023To describe specific clinical, multimodal imaging, and natural history features of an unusual variant of acute zonal occult outer retinopathy. (Observational Study)
Observational Study
PURPOSE
To describe specific clinical, multimodal imaging, and natural history features of an unusual variant of acute zonal occult outer retinopathy.
METHODS
Retrospective, observational, longitudinal, multicenter case series. Patients exhibiting this unusual clinical condition among cases previously diagnosed with acute zonal occult outer retinopathy were included. Multimodal imaging, laboratory evaluations, and genetic testing for inherited retinal diseases were reviewed.
RESULTS
Twenty eyes from 10 patients (8 females and 2 males) with a mean age of 54.1 ± 13.3 years (range, 38-71 years) were included. The mean follow-up duration was 13.1 ± 5.3 years (range, 8-23 years). Presenting symptoms were bilateral in 7 patients (85% of eyes) and included scotomata and photopsia. All patients had bilateral lesions at presentation involving the peripapillary and far peripheral retina. Baseline optical coherence tomography showed alteration of the retinal pigment epithelium and photoreceptor layers corresponding to zonal areas of fundus autofluorescence abnormalities. Centrifugal and centripetal progression of the peripapillary and far-peripheral lesions, respectively, occurred over the follow-up, resulting in areas of complete outer retinal and retinal pigment epithelium atrophy.
CONCLUSION
Initial alteration of photoreceptors and retinal pigment epithelium and a stereotypical natural course that includes involvement of the far retinal periphery, characterize this unusual condition. It may represent a variant of acute zonal occult outer retinopathy or may be a new entity. We suggest to call it multizonal outer retinopathy and retinal pigment epitheliopathy .
Topics: Adult; Aged; Female; Humans; Male; Middle Aged; Fluorescein Angiography; Retinal Diseases; Retinal Pigments; Retrospective Studies; Scotoma; Tomography, Optical Coherence; Visual Fields
PubMed: 37748093
DOI: 10.1097/IAE.0000000000003927 -
Klinische Monatsblatter Fur... Sep 2023There are only about 100 case reports on the Acute Idiopathic Blind Spot Enlargement Syndrome (AIBSES). This is characterised by the eponymous visual field loss in the...
There are only about 100 case reports on the Acute Idiopathic Blind Spot Enlargement Syndrome (AIBSES). This is characterised by the eponymous visual field loss in the blind spot area, acute onset photopsia, and funduscopically little or no change in the optic disc area, with conspicuous outer retinal bands on optical coherence tomography (OCT). Typical is the unilateral occurrence. Predominantly young women are affected. While previous reviews of AIBSES either predate the introduction of OCT or focus on differentiation from potentially related outer retinal conditions (e.g., multiple evanescent white dot syndrome and acute zonal occult outer retinopathy), the present review will concentrate on the current perspective and treatment strategies that have been developed and will aim to help increase awareness. Since the first description of AIBSES in the late 1980s, the introduction of OCT has simplified the diagnosis and characterisation of AIBSES as a disease of the outer retina. Nevertheless, misdiagnosis remains common in the spectrum of optic neuritis, as AIBSES may be ignored in differential diagnosis.
PubMed: 37678401
DOI: 10.1055/a-2130-5131