-
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 -
BMJ Case Reports Apr 2024Multiple evanescent white dot syndrome (MEWDS) is a rare inflammatory eye condition. We report an atypical case of MEWDS in a man in his 30s who presented with blurred...
Multiple evanescent white dot syndrome (MEWDS) is a rare inflammatory eye condition. We report an atypical case of MEWDS in a man in his 30s who presented with blurred vision (visual acuity 6/9), floaters and photopsia in his left eye. Funduscopy examination showed mild peripheral nasal vascular sheathing with subtle grey-white dots highlighted on fundus autofluorescence. As far as the authors are aware, this is the first case presentation whereby areas affected by MEWDS started in the peripheral retina and migrated centrally. Fluorescein angiography showed hyperfluorescent areas in wreath-like patterns nasally. Optical coherence tomography showed disruption of the ellipsoid zone and hyperreflective projections into the outer nuclear layer. The size of the involved area increased over 3 weeks and subsequently resolved over 4 months. Simultaneously, the patient's symptoms also resolved, without treatment. This case highlights the importance of multimodal imaging, especially ultrawidefield imaging in diagnosing MEWDS.
Topics: Humans; Male; Fluorescein Angiography; Tomography, Optical Coherence; Adult; Retinal Diseases; Visual Acuity; White Dot Syndromes; Retina; Syndrome
PubMed: 38684347
DOI: 10.1136/bcr-2023-255522 -
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 -
Retinal Cases & Brief Reports Jan 2024To describe multimodal imaging findings in a patient with a rare, symptomatic fundus lesion arising from the retinal pigment epithelium.
PURPOSE
To describe multimodal imaging findings in a patient with a rare, symptomatic fundus lesion arising from the retinal pigment epithelium.
METHODS
Case report.
RESULTS
A 36-year-old woman presented with photopsia in her left eye. Funduscopy revealed an 8-mm × 7-mm, dark brown lesion at the level of the retinal pigment epithelium inferior to the macula. The lesion had an irregular, cauliflower-like border and a light grey subretinal pseudohypopyon. On fundus autofluorescence, the lesion was markedly hypoautofluorescent with an irregular hyperautofluorescent margin. It was generally hyopofluorescent on fluorescein angiography and moderately hypofluorescent on indocyanine green angiography. Spectral-domain optical coherence tomography revealed a fine layer of subretinal fluid over the entire lesion, thinning of the outer retinal layers with loss of photoreceptors, and an irregular retinal pigment epithelium. Multiple drusen-like subretinal deposits were located along the lesion margin, and inferiorly, the pseudohypopyon appeared as a hyperreflective subretinal mass. During 3 years of follow-up, her symptoms remained unchanged and fundus photography showed minimal enlargement of the lesion.
CONCLUSION
Multimodal imaging findings of a solitary pigmented retinal pigment epithelium lesion with pseudohypopyon and subretinal fluid are shown. The lesion may represent an atypical variant of unilateral retinal pigment epithelium dysgenesis.
Topics: Female; Humans; Adult; Retinal Pigment Epithelium; Subretinal Fluid; Retina; Eye Diseases; Fluorescein Angiography; Tomography, Optical Coherence
PubMed: 35993774
DOI: 10.1097/ICB.0000000000001317