Occlusive Vasculopathy Research Articles

Reperfusion of macular vasculature in lupus-mediated occlusive vasculopathy

Reperfusion of macular vasculature in lupus-mediated occlusive vasculopathy

Combination therapy for Sneddon syndrome to reduce the incidence of cerebrovascular complications.

Sneddon syndrome is an occlusive vasculopathy that presents clinically with generalized livedo racemosa on the skin and transient ischemic attacks, strokes, and cognitive or motor deficits in the central nervous system. Antiplatelet or anticoagulant therapy is recommended. Due to the limited therapeutic efficacy and the resulting serious complications, we propose combination therapy with additional infusion cycles of alprostadil and captopril and report initial long-term results. We performed a systematic retrospective analysis of all patients with primary Sneddon syndrome who received combination therapy in our clinic between 1995 and 2020. Therapeutic outcomes were evaluated using descriptive statistics compared to historical controls receiving monotherapy. We also analyzed the event rate of complications when combination therapy was discontinued. During the 99.7 patient-years of follow-up, there were no transient ischemic attacks and the stroke rate dropped to 0.02 per patient-year. In comparison, the rates of transient ischemic attacks and strokes in the historical controls ranged from 0.08 to 0.035 per patient-year. After discontinuation of alprostadil therapy, eight events occurred in three patients. Combination therapy reduces the long-term incidence of ischemic events in patients with primary Sneddon syndrome.

Retinal toxicity secondary to inadvertent subretinal mitomycin C injection

Purpose: To report a patient who developed retinal toxicity following inadvertent subretinal mitomycin C injection during glaucoma surgery. Methods: Case report and review of the literature. Results: A 73-year-old man underwent glaucoma tube shunt surgery with sub-Tenon’s mitomycin C injection. Postoperatively he developed retinal edema, hemorrhages, and occlusive vasculopathy localized to the quadrant of the mitomycin C injection. It was determined that the sclera had been inadvertently punctured during mitomycin C injection, resulting in subretinal placement of the medication and resultant retinal toxicity. At postoperative month one, there was diffuse chorioretinal atrophy in the area of the injection. The macula was spared and he retained his preoperative visual acuity. Conclusion: Inadvertent subretinal mitomycin C injection may occur when attempting to inject the drug into the sub-Tenon’s space and can result in localized retinal toxicity.

Occlusive cutaneous vasculopathies as cause of chronic ulcers.

The term occluding vasculopathies covers a large number of different conditions. These often manifest as skin ulcers. Occluding vasculopathies should be considered in the differential diagnosis of leg ulcers. The term "occlusive vasculopathies" encompasses pathophysiologically related entities that share structural or thrombotic obliteration of small cutaneous vessels. In this article, we will focus on livedoid vasculopathy with and without antiphospholipid syndrome and calciphylaxis with differentiation from hypertonic leg ulcer as the most relevant differential diagnoses of leg ulcer. The term also includes vascular occlusion, for example due to oxalate or cholesterol embolism, and septic vasculopathy. This often leads to acral ulceration and is therefore not a differential diagnosis with classic leg ulcers. It will not be discussed in this article. Occlusive vasculopathy may be suspected in the presence of the typical livedo racemosa or (non-inflammatory) retiform purpura as a sign of reduced cutaneous perfusion in the wound area. Inflammatory dermatoses, especially vasculitides, must be differentiated. This is achieved by histopathological evaluation of a tissue sample of sufficient size and depth taken at the appropriate time. In addition, specific laboratory parameters, particularly coagulation parameters, can support the diagnosis.

MANAGEMENT EALES’ DISEASE GRADE III B: A RARE CASE

Introduction : Eales' disease is an idiopathic occlusive retinal vasculopathy that mostly affects the peripheral retina bilaterally in young, predominately male patients. A hallmark of eales disease is recurrent vitreous hemorrage can all cause some patients to experience severe vision loss
 Case Illustration : A 39-year-old male with vitreous hemorrhage caused by Eales Disease grade III B with initial visual acuity 1/300. Ultrasound imaging showed the membrane like lesion with 1/2 retinal reflectivity, after moderate movement and vitreous hemorrhage on both eyes. The photo-fundus showed there is decreased fovea reflex and perimacular exudate with visible contours of sclerotic blood vessels, visible cotton wall spot, and macroaneurysm. There is also haze grade 3 on media, visible folds in the inferior part, visible shadows of cotton wall spots. The patient was managed with pars plana vitrectomy with endolaser
 Discussion : In this case, patient was diagnosed as vitreous hemorrhage caused by Eales Disease grade III B on both eyes but it getting worse on left eye further. We chose to perform PPV with endolaser on right eye. The outcome was good on right eye based on better visual acuity and decreased hemorrhage, but in the left eye, which not treated, worsen outcome occurred
 Conclusion : The management of this patient’s case showed good results in both anatomic and functional outcomes for right eye, but unfortunately left eye is on serious problem

Retinal Vasculitis After Intravitreal Pegcetacoplan: Report From the ASRS Research and Safety in Therapeutics (ReST) Committee.

Purpose: To analyze post-marketing cases of retinal vasculitis after intravitreal pegcetacoplan. Methods: The American Society of Retina Specialists (ASRS) Research and Safety in Therapeutics (ReST) Committee as well as an expert panel performed a retrospective review of cases of retinal vasculitis reported to the ASRS. Clinical and imaging characteristics were reviewed for evidence of retinal vasculitis and analyzed. Results: Fourteen eyes of 13 patients were confirmed to have retinal vasculitis by review of imaging studies. All cases occurred after the first pegcetacoplan injection. Occlusive retinal vasculopathy was confirmed in 11 eyes (79%). Patients presented a median of 10.5 days (range, 8-23 days) after pegcetacoplan injection. All eyes had anterior chamber inflammation, and 12 eyes (86%) had vitritis. Vasculopathy involved retinal veins (100%) more than arteries (73%), and 12 eyes (86%) had retinal hemorrhages. The median visual acuity (VA) was 20/60 (range, 20/30-5/200) at baseline, 20/300 (range, 20/100-no light perception [NLP]) at vasculitis presentation, and 20/200 (range 20/70-NLP) at the last follow-up. Eight eyes (57%) had more than a 3-line decrease in VA, and 6 eyes (43%) had more than a 6-line decrease in VA from baseline to the final follow-up, including 2 eyes that were enucleated. Six eyes (43%) developed signs of anterior segment neovascularization. Conclusions: There is currently no known etiology for vasculitis in this series. Optimum treatment strategies remain unknown. Infectious etiologies should be considered, and corticosteroid treatments may hasten resolution of inflammatory findings. Continued treatment of affected patients with pegcetacoplan should be avoided.

Necrotic Ulcers from Occlusive Vasculopathy Associated with Monoclonal B Cell Lymphocytosis.

Abstract is missing (Shot communication)

Rare cause of occlusive retinal vasculopathy

Rare cause of occlusive retinal vasculopathy

Punch grafting for the treatment of ulcerated atrophie blanche.

Case report: A 79-year-old woman presented with a large painful ulcer on the lateral aspect of her left leg over a 6-month period and was diagnosed of ulcerated atrophie blanche. On an outpatient basis punch grafting was performed and 3weeks after, complete epithelization was achieved. Discussion: Ulcerated atrophie blanche is a misdiagnosed disorder with painful lesions and, consequently, a high impact on quality of life. Atrophie blanche describes porcelain-white colored, red-dotted atrophic plaques on legs or feet. It may be due to multiple causes, usually associated with alterations in the microcirculation. All causes of atrophie blanche can be included in the term livedoid vasculopathy, a type of occlusive vasculopathy without vasculitis. Many patients with atrophie blanche and livedoid vasculopathy have also chronic venous insufficiency. Etiological treatment should be prescribed in order to avoid progression of the lesions. In case of chronic venous insufficiency, control of venous hypertension is essential. Without anti-edema measures, superficial, very painful, and resistant ulcers may appear. These ulcers can be considered a wound on scar tissue; therefore, it must be treated as a hard-to-heal wound. As we show in this case, punch grafting is an effective therapeutic alternative for wound closure and pain reduction of ulcerated atrophie blanche.

Lupus mastitis and antiphospholipid syndrome treated with anticoagulation and immunosuppression: a case report

BackgroundSystemic lupus erythematosus is an autoimmune disease that can have cutaneous and systemic manifestations. Lupus panniculitis, also known as lupus mastitis, is a subset of chronic cutaneous lupus erythematosus that involves inflammation of the subcutaneous fat. The pathogenesis of lupus mastitis is not fully understood. Diagnosis involves a combination of skin manifestations, imaging, and pathologic confirmation. Treatment typically includes steroids and antimalarials, with more severe disease requiring additional immunosuppressive medications. This report highlights a case of lupus mastitis treated with rituximab and a possible relationship between this disease process and thrombotic disease.Case presentationA 48-year-old African American female with systemic lupus erythematosus and antiphospholipid syndrome presented with new breast lesion. Mammography revealed calcifications and increased density with coarse trabecular pattern. Breast biopsy showed features of cutaneous lupus and occlusive vasculopathy. The patient was diagnosed with lupus mastitis and treated with anticoagulation, rituximab, mycophenolate mofetil, and quinacrine with resolution of her symptoms.ConclusionThis patient experienced improvement in her breast symptoms with combination therapy including rituximab. There are only two other cases reported in literature of patients with lupus mastitis responding to rituximab, highlighting the possible role of B cell depleting therapy for those who have contraindications to standard treatments for lupus mastitis. While the pathophysiology of lupus mastitis is thought to be immune driven, some literature suggests that associated thrombosis commonly seen may be due to a physiologic overlap similar to antiphospholipid syndrome. The possible relationship between antiphospholipid syndrome and lupus mastitis and the use of antiplatelet and anticoagulation therapy is discussed and may warrant further investigation.

Eales Disease

Eales disease, first described by Henry Eales in 1880, constitutes an idiopathic occlusive retinal vasculopathy apparently multifactorial, and with a likely immunologic origin, which mainly affects the peripheral retina bilaterally in young patients predominantly males. Most cases are asymptomatic in the early stages of the disease however, in the course of the disease floaters and blurry vision arise and hemorrhages lead to significant visual loss. The clinical appearance depends on the peripheral nonperfusion and neovascularization that follow retinal perivasculitis. Visual loss is commonly due to vitreous hemorrhage and tractional retinal detachment. Routine biochemical tests, fundus fluorescein angiography, widefield angiography, optical coherence tomography, optical coherence tomography angiography, and ultrasound are used in the diagnosis of the disease. Corticosteroids, immunosuppressive agents, laser photocoagulation, intravitreal triamcinolone, anti-VEGF injections, and vitrectomy are treatment modalities.

Ischemic Stroke due to Intracerebral Steal from Calvarial Metastasis: a Case Report (P2-5.001)

<h3>Objective:</h3> NA <h3>Background:</h3> Malignancy is an important risk factor for ischemic stroke. Various mechanisms may explain the association between cancer and ischemic stroke, including hypercoagulation, accelerated atherosclerosis, and toxic effects of chemotherapy and radiation. Here, we report a rare case in which a patient with known occlusive radiation vasculopathy (ORV) presented with ischemic strokes caused by an “intracerebral steal” phenomenon from new metastatic calvarial lesions. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 64-year-old woman with remote history of left neck squamous cell carcinoma and prior left frontal stroke from left common carotid artery (CCA) occlusion secondary to ORV presented to the emergency department with acute-onset right hemiparesis. Brain magnetic resonance imaging revealed scattered infarcts in the convexity of the left hemisphere as well as adjacent calvarial destruction. On functional and structural MRI, the calvarial lesion demonstrated increased vascularity and diffusion restriction. CT angiography redemonstrated complete occlusion of the left CCA and strong right-to-left collateral circulation across the anterior communicating artery. The remainder of the stroke workup, including echocardiogram, laboratory analyses, venous dopplers, and carotid ultrasound was unrevealing. CT scans of the chest, abdomen and pelvis revealed only lytic lesions in the T11 vertebra, left ilium and right sacrum, and biopsy of the latter revealed adenocarcinoma. <h3>Conclusions:</h3> In this patient, neck radiation therapy had resulted in a chronic occlusion of the left CCA. Years later, a hypervascular skull metastasis drew from this limited blood supply, causing ischemic strokes in the parenchyma adjacent to the hypermetabolic calvarial metastasis. This case of an “intracerebral steal” phenomenon demonstrates how hypervascular extradural lesions may also lead to ischemia, particularly in cases of ipsilateral poor hemodynamic reserve. <b>Disclosure:</b> Miss Murthy has nothing to disclose. Dr. Kumar has nothing to disclose. Dr. Testai has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Swanson Martin &amp; Bell, LLP. Dr. Testai has received publishing royalties from a publication relating to health care. Edward Michals has stock in Off Color Brewing. Edward Michals has received personal compensation in the range of $100,000-$499,999 for serving as a Staff Physician with University of Illinois.

Bilateral Proliferative Retinopathy as the Initial Presentation of Atypical Hemolytic Uremic Syndrome: A Case Report.

In this case report, we describe a 34-year-old male patient who presented with vision loss and was found to have profound occlusive retinal vasculopathy. His initial laboratory studies were unremarkable, but five weeks after his ocular symptoms began, he developed acute multi-organ failure and was ultimately diagnosed with atypical hemolytic uremic syndrome (aHUS). His course was complicated by a stroke, respiratory distress requiring intubation, long-term hemodialysis, and eventually death. Occlusive retinal vasculopathy may be the presenting finding in aHUS, although thrombotic microangiopathy syndromes typically present with acute kidney injury and or failure, hemolytic anemia, and thrombocytopenia. [Ophthalmic Surg Lasers Imaging Retina 2023;54:297-300.].

Endothelial phosphoinositide 3-kinase-β inactivation confers protection from immune-mediated vascular injury.

Heart transplant and recipient survival are limited by immune cell-mediated injury of the graft vasculature. We examined the role of the phosphoinositide 3-kinase-β (PI3Kβ) isoform in endothelial cells (EC) during coronary vascular immune injury and repair in mice. In minor histocompatibility-antigen mismatched allogeneic heart grafts, a robust immune response was mounted to each wild-type, PI3Kβ inhibitor-treated, or endothelial-selective PI3Kβ knockout (ECβKO) graft transplanted to wild-type recipients. However, microvascular EC loss and progressive occlusive vasculopathy only developed in control, but not PI3Kβ-inactivated hearts. We observed a delay in inflammatory cell infiltration of the ECβKO grafts, particularly in the coronary arteries. Surprisingly, this was accompanied by an impaired display of proinflammatory chemokine and adhesion molecules by the ECβKO ECs. In vitro, tumor necrosis factor α-stimulated endothelial ICAM1 and VCAM1 expression was blocked by PI3Kβ inhibition or RNA interference. Selective PI3Kβ inhibition also blocked tumor necrosis factor α-stimulated degradation of inhibitor of nuclear factor kappa Bα and nuclear translocation of nuclear factor kappa B p65 in EC. These data identify PI3Kβ as a therapeutic target to reduce vascular inflammation and injury.

Case report of intracranial large vessel occlusion in glioblastoma multiforme patient after radiation therapy.

Vasculopathy of the large arteries is a prominent complication of radiotherapy. Radiation-induced cerebral vasculopathy can cause arterial stenosis/occlusion, cerebral hemorrhage, and aneurysm formation. We report a cases of glioblastoma multiforme (GBM) with occlusive radiation vasculopathy (ORV). This 28-year-old patient who suffered from GBM had surgery for cytoreduction and received postoperative CCRT. We adopted the radiotherapy and oncology group radiation guideline. This patient had cerebrovascular accident episodes without any known risk. Therefore, ORV was highly suspected and vascular stenosis was confirmed using magnetic resonance angiography (MRA) or digital subtraction angiography. Extracranial-intracranial bypass was performed and patency was confirmed. The patient had not suffered from recurrent symptoms of transient ischemic attack or ischemic stroke for 1.5 years. This is the first article to report bypass surgery for GBM patients. Although the median survival rate of GBM is approximately 15 months, the short survival time may be sufficient for occlusive vasculopathy to occur. Regular follow-up magnetic resonance imaging assessments are recommended, as is MRA as a screening tool for the early diagnosis of ORV.The Stenting versus Aggressive Medical Management for Preventing Recurrent Stroke in Intracranial Stenosis (SAMMPRIS) trial focused on atherosclerotic intracranial arterial stenosis, revealing that aggressive medical management was superior to stenting for secondary stroke prevention; however, it did not mention radiation-induced vasculopathy. Bypass surgery has yielded some positive outcomes. In the absence of contraindications, antiplatelet or anticoagulation agents could be added, and bypass surgery could be performed because there was no stent in the distal intracranial arteries. MRA is a potential screening tool for ORV in GBM patients and bypass surgery could be performed to improve brain perfusion. Bypass surgery could help patient with occlusive radiation vasculopathy.

The Eye as a Window to the Brain: Prominent Retinal Vasculopathy Points to Neuro-Behcet Diagnosis for an Undifferentiated Solitary Brain Lesion

Objective To report a perplexing case of Behcet disease (BD) presenting as a focal parenchymal lesion that reached a diagnosis after noting a prominent retinal vasculopathy, highlighting the importance of ophthalmologic evaluation in undifferentiated CNS disease. Background BD can have variable systemic manifestations driven by a vasculitis, including oral or genital ulcers, pulmonary aneurysms, and uveitis. Neurologic involvement is present in less than 10% of patients, most commonly as a meningoencephalitis. Design/Methods We present the case of a woman who developed asymmetric sub-acute sensorineural hearing loss at age 31 followed by transient right facial weakness at age 40, and most recently presented with right facial numbness and arm weakness at age 47. Brain MRI revealed a left frontal enhancing lesion with associated T2/FLAIR hyperintensity extending from the periventricular to the juxtacortical area with a thin rim of a reduced diffusion. CSF and serum studies were negative for inflammation, infection and malignancy except for elevated ESR and CRP. Brain biopsy revealed non-specific gliosis. Persistent enhancement on MRI was noted over 3 months, with spontaneous clinical improvement. Patient endorsed insidious vision changes over recent years, and visual testing was performed. Results Dilated ophthalmic examination demonstrated striking peripheral attenuation and sclerosis of retinal vasculature, with evidence of nonperfusion and skip lesions on retinal fluorescein angiography (FA). Findings of occlusive retinal peripheral vasculopathy suggested an underlying vasculitis as the etiology of the brain lesion and prior deficits, raising the likelihood of BD. Patient was homozygous for HLA-B*51, further supporting this diagnosis even with lack of mucosal ulcers and negative history of pathergy. Conclusions Neurologic manifestations of BD can be diverse including retinal occlusive vasculopathy; ulcers are not universally present. Ophthalmologic examination, even when minimally symptomatic, can inform the diagnosis of CNS lesions. Patient was started on Prednisone, Infliximab and Methotrexate, achieving disease remission.

Rapidly progressing dyspnea and retiform purpura

Rapidly progressing dyspnea and retiform purpura

Choroidal occlusive vasculopathy after intraarterial chemotherapy: MRI findings

PurposeTo evaluate magnetic resonance imaging (MRI) findings in patients suffering choroidal occlusive vasculopathy (COV) after intra-arterial chemotherapy (IAC) for retinoblastoma. MethodsA retrospective study of 37 eyes of 34 patients receiving IAQ between 2016 to 2021 as primary or secondary treatment for retinoblastoma was conducted. Twenty-two patients received systemic chemotherapy with carboplatin, vincristine and etoposide. The rest received IAC as primary treatment. The drugs administered were melphalan (3−4mg), carboplatin (40mg) plus topotecan (20mg). The patients were examined under general anaesthesia every month to observe tumor regression and possible complications of the treatment. For the patients with COV an MRI was obtained to analyse the choroidal thickness and axial ocular length. ResultsA COV was observed in 5 of the 37 eyes receiving IAC (13,51%), all of them with a complete sectorial choroidopathy not sparing the fovea (grade 2). In 4 of the 5 patients the choroidal thickness was decreased and in three cases the size of the eye which presented COV was clearly smaller than the contralateral eye. Tumor control was archived in all 5 patients. ConclusionIn our cases COV was associated with reduction of thinning of choroid and eye length in the MRI. A new classification maybe needed to correlate better with the severity of the complication affecting the fovea. Although early results generally are favorable to the use of IAC, longer follow up and scrupulous documentation of side effects will be necessary to know the true role of IAC for retinoblastoma.

Perinatal hypoxia aggravates occlusive pulmonary vasculopathy in SU5416/hypoxia-treated rats later in life.

Pulmonary arterial hypertension (PAH) is a fatal disease, which is characterized by occlusive pulmonary vascular disease (PVD) in small pulmonary arteries. It remains unknown whether perinatal insults aggravate occlusive PVD later in life. We tested the hypothesis that perinatal hypoxia aggravates PVD and survival in rats. PVD was induced in rats with/without perinatal hypoxia (embryonic day 14 to postnatal day 3) by injecting SU5416 at 7 wk of age and subsequent exposure to hypoxia for 3 wk (SU5416/hypoxia). Hemodynamic and morphological analyses were performed in rats with/without perinatal hypoxia at 7 wk of age (baseline rats, n = 12) and at 15 wk of age in 4 groups of rats: SU5416/hypoxia or control rats with/without perinatal hypoxia (n = 40). Pulmonary artery smooth muscle cells (PASMCs) from the baseline rats with/without perinatal hypoxia were used to assess cell proliferation, inflammation, and genomic DNA methylation profile. Although perinatal hypoxia alone did not affect survival, physiological, or pathological parameters at baseline or at the end of the experimental period in controls, perinatal hypoxia decreased weight gain and survival rate and increased right ventricular systolic pressure, right ventricular hypertrophy, and indices of PVD in SU5416/hypoxia rats. Perinatal hypoxia alone accelerated the proliferation and inflammation of cultured PASMCs from baseline rats, which was associated with DNA methylation. In conclusion, we established the first fatal animal model of PAH with worsening hemodynamics and occlusive PVD elicited by perinatal hypoxia, which was associated with hyperproliferative, proinflammatory, and epigenetic changes in cultured PASMCs. These findings provide insights into the treatment and prevention of occlusive PVD.

Vasculopatía coroidea oclusiva tras quimioterapia intraarterial: hallazgos en la RMN

PurposeTo evaluate magnetic resonance imaging (MRI) findings in patients suffering choroidal occlusive vasculopathy (COV) after intra-arterial chemotherapy (IAC) for retinoblastoma. MethodsA retrospective study of 37 eyes of 34 patients receiving IAQ between 2016 to 2021 as primary or secondary treatment for retinoblastoma was conducted. Twenty-two patients received systemic chemotherapy with carboplatin, vincristine and etoposide. The rest received IAC as primary treatment. The drugs administered were melphalan (3-4mg), carboplatin (40mg) plus topotecan (20mg). The patients were examined under general anaesthesia every month to observe tumor regression and possible complications of the treatment. For the patients with COV an MRI was obtained to analyse the choroidal thickness and axial ocular length. ResultsA COV was observed in 5 of the 37 eyes receiving IAC (13,51%), all of them with a complete sectorial choroidopathy not sparing the fovea (grade 2). In 4 of the 5 patients the choroidal thickness was decreased and in three cases the size of the eye which presented COV was clearly smaller than the contralateral eye. Tumor control was archived in all 5 patients. ConclusionIn our cases COV was associated with reduction of thinning of choroid and eye length in the MRI. A new classification maybe needed to correlate better with the severity of the complication affecting the fovea. Although early results generally are favorable to the use of IAC, longer follow up and scrupulous documentation of side effects will be necessary to know the true role of IAC for retinoblastoma.