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Systemic lupus erythematosus and ocular involvement: an overview.

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology and with remarkably heterogeneous clinical feat … rtial occlusion of the lacrimal punctum by thermal cautery is rarely applied. Although uncommon, episcleritis and scleritis can sometimes be detected as an initial finding of SLE and reveal themselves as moderate to intense ocular pain, redness, blurred vision, and lacrimation. Unilateral or more often bilateral retinopathy is responsible for visual loss of …

Adverse Reaction to COVID-19 mRNA Vaccination in a Patient With VEXAS Syndrome.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a rare genetic disorder originating from a somatic mutation in the hematopoietic stem cells. This syndrome was first described in 2020 and carries many clinical features that other conditions cannot explain. Widespread autoinflammation is the primary process the disease presents, with high morbidity and mortality in those who show signs of bone marrow failure. Treatment is complex, and response to current therapies is poor. Long-term prognosis carries a mortality of 50%. In addition, the advancement of new-generation

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… Image D: shows scleritis with associated facial swelling. Figure 2 Ruptured bulla on the right arm at the injection site. … Prior MRI of the orbits was consistent with an intraconal/retro-orbital fat tracking proximally along the optic nerve sheath to the orbital apex and right scleritis with right superior rectus muscle involvement. …

Analysis of a novel protocol of pulsed intravenous cyclophosphamide for recalcitrant or severe ocular inflammatory disease.

PURPOSE: To analyze the success rate of pulsed intravenous (IV) cyclophosphamide (CyP) for noninfectious ocular inflammatory disease and to identify risk factors for failure of therapy. DESIGN: Retrospective, interventional, noncomparative cohort study. PARTICIPANTS: One hundred ten eyes of 65 patients. METHODS: Through a computer search of the Massachusetts Eye Research and Surgery Institution's database, we identified patients who were treated with IV CyP between May 2005 and April 2012. We obtained demographic and clinical information through review of the electronic health record of eac

Relapsing polychondritis in the Department of Defense population and review of the literature.

OBJECTIVE: The objective of this study was to characterize the clinical features of relapsing polychondritis (RPC) within the Department of Defense beneficiary population and determine the utility of echocardiography, imaging studies, and pulmonary function testing for diagnosis and monitoring disease. METHODS: We performed a retrospective Electronic Medical Record chart review of all patients diagnosed with RPC within the Department of Defense between January 2004 and December 2009. RESULTS: Thirty patients met McAdam's diagnostic criteria and an additional 13 met our criteria for partial R

Safety and tolerability of zoledronic acid and other bisphosphonates in osteoporosis management.

Bisphosphonates (BPs) are widely used in the treatment of postmenopausal osteoporosis and other metabolic bone diseases. They bind strongly to bone matrix and reduce bone loss through inhibition of osteoclast activity. They are classified as nitrogen- and non-nitrogen-containing bisphosphonates (NBPs and NNBPs, respectively). The former inhibit farnesyl diphosphate synthase while the latter induce the production of toxic analogs of adenosine triphosphate. These mechanisms of action are associated with different antifracture efficacy, and NBPs show the most powerful action. Moreover, recent evi

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… However, the true incidence of these side effects is unknown. 72 , 73 A large cohort study determined the six-month incidence of uveitis/scleritis during BP treatment. … The relative risk of uveitis/scleritis over 6 months was 1.23 (95% confidence interval [CI] 0.85–1.79) compared with patients not treated with BPs. …

Ocular manifestations of coronavirus disease 2019.

PURPOSE: Coronavirus disease 2019 (COVID-19) is an ongoing global public health problem, and most of the COVID-19 research is focused mainly … a (n = 9), increased secretion (n = 6), chemosis (n = 3), follicular conjunctivitis (n = 2), and episcleritis (n = 2). The most common symptom was photophobia (n 15). Patients with ocular involvement were more likely to have higher neutrophil counts (p = 0.001), and increased CRP (p < 0.001), PCT (p = 0.001), and ESR levels (p < 0.001). Mean lymphocyte coun …

A Novel NOD2-associated Mutation and Variant Blau Syndrome: Phenotype and Molecular Analysis.

PURPOSE: To describe the clinical and molecular implications of a novel mutation in the NOD2/CARD15 gene on a family and its seven affected members. METHODS: We reviewed the clinical presentations of family members who came to our center for refractory uveitis. Genetic testing and molecular testing was performed. RESULTS: All affected members had adult onset recurrent non-granulomatous panuveitis. The inheritance pattern suggested an autosomal dominant disease and genetic analysis identified a novel mutation in the NOD2 gene that converted amino acid 600 from glutamate to alanine (E600A). Tr

Окулярная токсичность целевой терапии и иммунотерапии гематологов.

Целенаправленная терапия и иммунная терапия изменили методы борьбы с гематологическими заболеваниями. Однако эти агенты могут также иметь непреднамеренные окулярные неблагоприятные последствия. Эти токсичные вещества часто недооцениваются, но могут существенно повлиять на качество жизни пациентов и на процесс принятия терапевтических решений. Всеобъемлющее понимание этих последствий имеет важное значение для междисциплинарного управления. В этом обзоре обобщаются имеющиеся в настоящее время данные об окулярной токсичности, связанной с современными целенаправленными и иммунными терапиями, испол

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… Keratitis and scleritis/episcleritis constitute about 10–11% of reported cases ( 12 ). For example, cases of peripheral ulcerative keratitis and corneal melts have occurred with ICIs, albeit rarely. … Scleritis or keratitis from ICIs is managed with topical steroids and sometimes systemic NSAIDs or steroids, similar to idiopathic cases. In refractory ocular inflammation, steroid-sparing immunosuppressants (such as infliximab for uveitis, or mycophenolate) have been used. …

Targeting the P-selectin/PSGL-1 pathway: discovery of disease-modifying therapeutics for disorders of thromboinflammation.

P-selectin is a membrane glycoprotein and a member of the selectin family of cell adhesion molecules. It is prestored in α-granules of platelets and Weibel-Palade bodies of endothelial cells and is rapidly expressed on their surfaces upon activation during the course of an inflammatory response. Although a critical component of the innate immune system, the interaction of P-selectin with its cognate ligand, P-selectin glycoprotein ligand 1 (PSGL-1) may mediate maladaptive events central to the pathophysiology of venous thromboembolism, cardiovascular disease, stroke, metabolic syndrome, and si

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… NCT03860506 To evaluate the safety of an orally administered single dose in patients with scleritis Phase 1 Effective inhibition of leukocyte rolling in scleral blood vessels of individuals with autoimmune scleritis was not achieved NCT00367692 P-selectin expression inhibitors Famotidine 111 To assess … | Phase 1 | Effective inhibition of leukocyte rolling in scleral blood vessels of individuals with autoimmune scleritis was not achieved | NCT00367692 P-selectin expression inhibitors | | | | Famotidine 111 | To assess efficacy in reducing endothelial expression of P-selectin in children with SCD …

Difficulty of diagnosing Wegener's granulomatosis in the head and neck region.

OBJECTIVE: The objective of this study was to review the various clinical features associated with Wegener's granulomatosis (WG) in the head and neck region and to discuss the difficulty of diagnosing patients with early stage WG. METHODS: Between January 1998 and August 2007, WG was diagnosed and treated in 16 patients at the Department of Otolaryngology, Hyogo College of Medicine. Clinical and operating records of these patients were analyzed retrospectively. Diagnosis was based on the Japanese criteria proposed by the Japanese Ministry of Health and Welfare in 1998. RESULTS: Ten patients

Clinical role of bisphosphonate therapy.

Bisphosphonates (BPs) are synthetic analogues of pyrophosphate. They inhibit bone resorption and are therefore widely used in disorders where there are increases or disruptions in bone resorption. This includes postmenopausal osteoporosis, glucocorticoid-induced osteoporosis, Paget's disease of bone, and malignancy-related bone loss. To best understand the clinical application of BPs, an understanding of their pharmacokinetics and pharmacodynamics is important. This review describes the structure, pharmacology and mode of action of BPs, focusing on their role in clinical practice. Controversie

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… Several case reports 82 , 83 have highlighted the association between the use of oral BPs (alendronate and risedronate) and anterior uveitis and scleritis. In most of these case reports, severe eye pain occurred within days of taking oral BPs and resolved after discontinuation of the drugs. … Postmarketing surveillance rates of uveitis and scleritis with bisphosphonates among a national veteran cohort. Retina. 2008;28(6):889–893. doi: 10.1097/IAE.0b013e31816576ef. 85. Etminan M, Forooghian F, Maberley D. …

From post-war reconstruction to the twenty-first century - ophthalmic pathology in Freiburg 1945-2015. Part 2: review of 43,169 histological diagnoses from 39,256 specimens collected over 71 years at a large German tertiary eye care centre.

BACKGROUND: Ophthalmic pathology is essential for diagnosing ocular diseases, correlating clinical and histopathological findings, and advancing research. The Eye Center at the University of Freiburg, Germany, has archived histopathological specimens since 1945, offering a unique resource to analyse long-term diagnostic trends. This study examines 43,169 diagnoses from 39,256 specimens over 71 years (1945–2015), providing insights into the evolution of ophthalmic pathology at a major tertiary care centre. METHODS: We performed a retrospective analysis of all archived ophthalmic pathology repor

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… | 1 (100%) | Filtering bleb revision | 1 (33%) | Normal | 2 (50%) | Filtering bleb revision | 2 (29%) | Normal | 6 (60%) | Scleritis | 4 (31%) | | | | | Normal | 1 (33%) | Scleritis | 2 (50%) | Scleritis | 2 (29%) | Fistula | 1 (10%) | Normal | 2 (15%) | | | | | Sclerouveitis | 1 (33%) | … Sclera Among all the registered 38 scleral samples, the most frequent diagnosis was normal sclera (32%), which was removed during trabeculectomy, followed by scleritis (27%). …

Comorbidities in Black South Africans with established rheumatoid arthritis.

OBJECTIVE: Comorbidities contribute both to morbidity and mortality in rheumatoid arthritis (RA). The aim of the current study was to investigate the prevalence and spectrum of comorbidities in South Africans with established RA. METHODS: A retrospective, consecutive case record review of 500 Black South African patients with established disease of ≥5 years attending a tertiary rheumatology service was performed. Common comorbidities including those listed in the Charlson Comorbidity Score (CCS) were documented. RESULTS: Most patients, 463 known alive (AG) and 37 known deceased (DG), were fe

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… Anemia, nodulosis, scleritis, cutaneous vasculitis and interstitial lung disease (ILD) diagnosed by high‐resolution computed tomography, were recorded as extra‐articular manifestations. Autoantibodies included rheumatoid factor (RF) and anti‐citrullinated peptide antibodies. … | .15 American College of Rheumatology functional class 3/4 | 348 (69.6) | 312 (67.4) | 36 (97.3) | 17.42 (2.37, 128.29) | .0001 Extra‐articular disease Anemia | 391 (78.2) | 357 (77.0) | 34 (91.9) | 3.37 (1.01, 11.18) | .04 Nodulosis | 155 (31.0) | 137 (29.6) | 18 (48.6) | 2.25 (1.15, 4.43) | .02 Scleritis …

Association of PDGF-BB-induced thrombomodulin with the regulation of inflammation in the corneal and scleral stroma.

PURPOSE: The responses of corneal and scleral stromal cells to platelet-derived growth factor (PDGF)-BB were assessed and inflammatory reactions in the cornea and sclera were investigated. METHODS: Primary cultures of cells obtained from human subjects and strains derived from human corneal or scleral stromal cells (Cs3 and Sc1, respectively) were used. Changes in gene expression after 24 hours of exposure to 10 ng/mL PDGF-BB were analyzed with an Sc1 DNA microarray. The upregulation of several genes in Cs3 and Sc1 was confirmed by reverse transcription-polymerase chain reaction (RT-PCR) and

Primary neuroendocrine lung tumor presenting with acute ileal obstruction. Case report.

INTRODUCTION: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently identified, somatic mutation-driven, a … resent a case of a 69-year-old male with no notable medical history who initially presented with episcleritis progressing to complete ophthalmoplegia. CT findings showed marked proptosis of the left eye and thickening of the lateral rectus. Over the subsequent months he presented to hospital repeatedly with worsening fibrotic lung disease. Eventually, after …

VEXAS Syndrome-Review.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly defined refractory adult-onset autoinflammatory syndrome caused by somatic mutations in the ubiquitin-like modifier-activating enzyme 1 (UBA1) gene in hematopoietic stem and progenitor cells, resulting in a shift in UBA1 isoform expression. Thus, patients develop a spectrum of systemic inflammatory manifestations and hematologic symptoms. To date, patients respond poorly to immune suppressive drugs, except high-dose glucocorticoids, and no treatment guidelines have been established. Given the high mortality ra

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… | 11 (44) | | | | 6 (50) | 17 (15) | | | 4 (36) | Hearing loss | 10/16 (63) | 1 (9) | 7 (47) | | | | 24 (29) | 5 (50) | | Cochlea/vestibular dysfunction | | 1 (7) | | | | | | 1 (7) | Ocular involvement | 7 (28) | 5 (46) | | 6 (43) | 5 (42) | 47 (40) | 20 (24) | 4 (32) | 4 (36) | Scleritis … But no etiologic link between VEXAS and these disorders has been confirmed. 47 Other Clinical Manifestations Other rare clinical manifestations have been gradually reported, including ocular inflammation (periorbital edema, uveitis, scleritis), gastrointestinal infiltration (abdominal pain, chronic …

Relapsing polychondritis: tracheobronchial involvement and differential diagnoses.

This review is describing the diagnostic and therapeutic approach to tracheobronchial involvement in relapsing polychondritis (RP), with a focus on differential diagnoses of inflammatory origin. RP is a systemic auto-immune disease that mainly affects cartilage structures, progressing through inflammatory flare-ups between phases of remission and ultimately leading to deformation of the involved cartilages. Besides the damage of auricular or nasal cartilage, tracheobronchial and cardiac involvement are the most severe, and can seriously alter the prognosis. Tracheobronchial lesions are assesse

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… Various manifestations have been described, but the most common are episcleritis, scleritis, and conjunctivitis ( 15 - 18 , 21 ). … ❖ Orbital or periorbital edema, sometimes associated with scleritis, episcleritis, uveitis, or retinal vasculitis ( 6 - 8 , 46 - 48 ). ❖ Peripheral neurological system manifestations with sensitive neuropathy or multiple mononeuropathy ( 47 , 48 ). …

Extraskeletal benefits and risks of calcium, vitamin D and anti-osteoporosis medications.

UnlabelledDrugs used for the prevention and the treatment of osteoporosis exert various favourable and unfavourable extra-skeletal effects whose importance is increasingly recognized notably for treatment selection.IntroductionThe therapeutic armamentarium for the prevention and the treatment of osteoporosis is increasingly large, and possible extra-skeletal effects of available drugs could influence the choice of a particular compound.MethodsThe present document is the result of a national consensus, based on a systematic and critical review of the literature.ResultsObservational research has

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… Bisphosphonate and ocular risk Cases of iritis, episcleritis and scleritis, but also conjunctivitis, have been reported after therapy with n-BPs (mainly alendronate, pamidronate disodium and zoledronic acid) in up to 1% [ 145 – 147 ]. … In severe cases of uveitis and scleritis, it could be better to discontinue IV BP [ 149 ]. …

Multisystemic sarcoidosis-important lessons learnt from one of the great imitators.

We report a case of a woman who was admitted with a suspicion of metastatic malignancy of unknown primary origin. A few months prior to her admission, she presented to a rheumatologist with acute anterior uveitis, psoriasiform rashes and polyarthritis. A diagnosis of psoriatic arthropathy was made and she was treated accordingly. Soon after she presented with persistent back and right upper quadrant abdominal pain for which she had a CT scan done with evidence of hilar lymphadenopathy, liver hypodensities and lytic-sclerotic bone lesions. She was referred to our hospital for further investigat

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… Table 3 Clinical features of some select autoimmune disorders Condition | Classical clinical features Granulomatosis with polyangiitis 81 82 | Commonly occurs in older adults Prodromal: fever, malaise, anorexia, weight loss Recurrent sinusitis, epistaxis, scleritis (more common than uveitis), otitis … media, polychondritis, oral and/or nasal ulcers, cough, haemoptysis, abnormal chest radiograph (nodules, fixed infiltrates, cavities), abnormal urinary sediment (microscopic haematuria), leucocytoclastic angiitis, conjunctivitis, corneal ulcerations, scleritis, optic neuropathy, retinal vasculitis, and …