scholarly journals Severe Adult Still’s Disease Complicated by Purtscher-Like Retinopathy Treated with Intravenous Pulse Methylprednisolone and Tocilizumab

2021 ◽  
pp. 531-537
Author(s):  
Kaho Akiyama ◽  
Yukiko Iwasaki ◽  
Rie Tanaka
Keyword(s):  
Visual Field ◽  
Visual Field Defect ◽  
Oral Prednisolone ◽  
High Dose ◽  
Still’S Disease ◽  
Poor Prognostic Factor ◽  
Pulse Methylprednisolone ◽  
Still's Disease ◽  
Adult Still's Disease ◽  
Field Defect

Adult Still’s disease (ASD) is a rare systemic inflammatory disorder in which ocular manifestations have rarely been described. We report a 29-year-old Japanese woman with a rare case of refractory ASD complicated by Purtscher-like retinopathy. She was diagnosed with ASD and started on a high dose of oral prednisolone. Two days after the initiation of the treatment, she presented with blurred vision in the left eye, and the funduscopic examination revealed bilateral Purtscher-like retinopathy. Despite treatment with high-dose oral prednisolone for 2 weeks, she developed macrophage activation syndrome. Considering the severity of ASD, intravenous pulse methylprednisolone therapy and tocilizumab injection were administered. Although all the laboratory data and Purtscher-like retinopathy gradually improved, nerve fiber layer defect (NFLD) in both eyes appeared and visual field defect remained corresponding to the NFLD. In conclusion, Purtscher-like retinopathy might be useful as a poor prognostic factor of ASD, which needs appropriate systemic immunosuppressive treatment. Early detection and long-term follow-up of Purtscher-like retinopathy is important because it has the possibility of developing permanent visual field defect.

scholarly journals Infliximab-related Optic Neuritis in a Patient with Crohn's Disease: a Case Report and Literature Review

2021 ◽  
Vol 62 (8) ◽  
pp. 1160-1166
Author(s):  
Su Hwan Park ◽  
Min Seung Kang ◽  
Sang Yoon Kim ◽  
Ji-Eun Lee ◽  
Su Jin Kim
Keyword(s):  
Visual Acuity ◽  
Crohn’S Disease ◽  
Visual Field ◽  
Crohn's Disease ◽  
Optic Neuritis ◽  
Visual Field Defect ◽  
High Dose ◽  
Infliximab Treatment ◽  
Blurred Vision ◽  
Field Defect

Purpose: We report a case of optic neuritis related to infliximab treatment in a patient with Crohn’s disease, along with a review of the relevant literature.Case summary: A 22-year-male patient complained of blurred vision in the left eye for 2-3 weeks. His best-corrected visual acuity was 8/20 in the left eye. Relative afferent pupillary defect was detected in the left eye and the pupils were of equal size. There was no pain on ocular movement. The results of slit-lamp and fundus examinations were normal. A visual field test revealed a central to inferior visual field defect in the left eye. Orbit magnetic resonance imaging revealed perineural enhancement of the left optic nerve. He had a 5-year history of Crohn’s disease and had been treated with intravenous infliximab (600 mg every 2 weeks). A diagnosis of retrobulbar optic neuritis associated with infliximab was made. He was infused with high-dose methylprednisolone, which was changed to per oral administration. His visual acuity and visual field defect improved after 3 months.Conclusions: Tumor necrosis factor-α inhibitors, such as infliximab, may cause optic neuritis. Therefore, history-taking is important for differential diagnosis and appropriate treatment.

scholarly journals Non-purulent pharyngitis: adult still’s disease

2020 ◽  
Vol 8 (9) ◽  
pp. 3362
Author(s):  
Nagabhushanarao B. V. ◽  
Narmada Vatti ◽  
Naveen Kumar Dannana ◽  
Surendra Konathala
Keyword(s):  
Serum Ferritin ◽  
Severe Disease ◽  
Elevated Serum ◽  
Unknown Origin ◽  
High Dose ◽  
Still’S Disease ◽  
Delay In Diagnosis ◽  
Still's Disease ◽  
Adult Still's Disease ◽  
Cell Counts

 Adult Still’s disease is rare and may present as pyrexia of unknown origin. Due to lack of expertise, diagnosis may be delayed inadvertently. The patient usually presents with spiked fever, polyarthralgia or arthritis, evanescent skin rash, non-purulent pharyngitis, lymphadenopathy and hepatosplenomegaly. Leukocytosis, predominantly of neutrophils, elevated erythrocyte sedimentation rate and C-reactive protein without obvious infection are the hallmarks of the disease. Delay in diagnosis may expose the patient to the side effects of antibiotics as they are repeatedly prescribed in view of elevated leukocytes. The majority of patients report pain in the throat without evidence of infection. This was an important clue to our diagnosis of this patient. Grossly elevated serum ferritin is diagnostic of adult onset still's disease. As the white cell counts are grossly elevated, a bone marrow examination to rule out hematological malignancy may be mandatory. Serum ferritin value has prognostic value too. Minor illness may respond to non-steroidal anti-inflammatory drugs (NSAIDs), but steroids are the mainstay of the treatment.  Methotrexate is of additional value for those presenting predominantly with arthritis. Anakinra, Infliximab and Tocilizumab are other options. Those patients presenting with severe disease and organ involvement require high dose intravenous steroids followed by high dose oral steroids.

Visual Field Examination in Glaucoma Patients : Humphrey Visual Field Analysis vs Amsler Grid

2017 ◽  
Vol 43 (2) ◽  
pp. 124
Author(s):  
Ivana Tanoko ◽  
Fifin L Rahmi
Keyword(s):  
Visual Field ◽  
Visual Field Defect ◽  
Glaucoma Patient ◽  
Field Analysis ◽  
Glaucomatous Optic Neuropathy ◽  
Visual Field Examination ◽  
Cross Sectional ◽  
Rnfl Thickness ◽  
Amsler Grid ◽  
Field Defect

Introduction and Objective: Glaucoma is the leading cause of global irreversible blindness, signed by glaucomatous optic neuropathy related to visual field defect. The purpose of the study is comparing visual field defect examination using HVFA to Amsler Grid in glaucoma patient at dr. Kariadi Hospital. Methods: This is a cross-sectional study. Amsler Grid were performed to the patients who have reliable HVFA at last 6 months and presented as descriptive analytic results. Result: There were 40 eyes involved in this study from 27 patients (15 men, 12 women), 26-68 years old and visual acuity 1/60-6/6. Seventeen eyes showed visual field defect in HVFA and Amsler Grid had average MD - 24.97 dB, CDR 0.89 and RNFL thickness 51.74. We found that 11 eyes didn’t showed in both of examination had average MD -8.06, CDR 0.63 and RNFL thickness 103.23 and those parameters are significantly different to the 17 eyes before (p<0.05). Data from 12 eyes that showed visual field defect only one of examination (9 only in HVFA and 3 in Amsler Grid) didn’t show difference statistically each other. Conclusion: HFVA and Amsler Grid seemed to be comparable in detecting visual field defect in advanced glaucoma.

scholarly journals Acute Zonal Occult Outer Retinopathy: Vision Loss in an Active Duty Soldier

2013 ◽  
Vol 2013 ◽  
pp. 1-5
Author(s):  
Courtney M. Crawford ◽  
Bruce A. Rivers ◽  
Mark Nelson
Keyword(s):  
Visual Field ◽  
Visual Field Defect ◽  
Vision Loss ◽  
Fundus Autofluorescence ◽  
Final Diagnosis ◽  
Active Duty ◽  
Visual Field Defects ◽  
Fundus Photographs ◽  
Optical Coherence Tomograph ◽  
Field Defect

Objective. To describe a case of acute zonal occult outer retinopathy (AZOOR) in an active duty patient.Methods. In this paper we studied fundus photographs, optical coherence tomograph, Humphrey visual field 30-2, fundus autofluorescence images, fluorescein angiograms, and electroretinography.Results. Exam findings on presentation: a 34-year-old American Indian female presented with bilateral photopsias, early RPE irregularity, and an early temporal visual field defect. Progression RPE damage and visual field defect along with ERG findings support final diagnosis of AZOOR.Conclusion. AZOOR may initially be identified as a broader category of disease called the “AZOOR complex of disorders”. Specific visual field defects, ERG results, and clinical exam findings will help distinguish AZOOR from other similar disorders.

scholarly journals AB0660 COVID-19 SHARES CLINICAL FEATURES WITH ANTI-MELANOMA DIFFERENTIATION ASSOCIATED PROTEIN 5 POSITIVE DERMATOMYOSITIS AND ADULT STILL’S DISEASE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1362.1-1362
Author(s):  
Y. Kondo ◽  
Y. Kaneko ◽  
H. Takei ◽  
H. Tamai ◽  
T. Takeuchi
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Features ◽  
Serum Ferritin ◽  
Rheumatic Diseases ◽  
Macrophage Activation ◽  
Cytokine Storm ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Still's Disease

Background:The coronavirus disease 2019 (COVID-19), caused by a novel corona virus named SARS-CoV-2, has emerged as a global pandemic. Severe inflammatory process is one of main pathogenesis of COVID-19 and this involves cytokine storm along with overactivation of macrophage. On another front, cytokine storm with macrophage activation is frequently observed in various connective tissue diseases including dermatomyositis with positive antimelanoma differentiation-associated protein 5 (anti-MDA5) autoantibodies and adult Still’s disease. Macrophage activation during inflammatory states is partially characterized by an increased serum ferritin levels and hyperferritinaemia and characteristics shared by the three diseases are a topic of interest to rheumatologists, however, no study has evaluated anti-MDA5-positive dermatomyositis and adult Still’s disease in comparison to COVID-19.Objectives:The aim of this study was to highlight the homology and heterogeneity of COVID-19, anti-MDA5 dermatomyositis, and adult Still’s disease by comparing clinical pictures of each disease in order to discuss their respective pathogeneses.Methods:We reviewed consecutive, newly diagnosed, untreated patients with COVID-19, anti-MDA5 dermatomyositis, or adult Still’s disease. We compared their clinical, laboratory, and radiological characteristics, including the prevalence of macrophage activation syndrome and lung involvement in each disease.Results:The numbers of patients with COVID-19, anti-MDA5 dermatomyositis, and adult-onset Still’s disease with hyperferritinaemia (serum ferritin ≥ 500ng/dL) who were included for main analysis were 22, 14, and 59, respectively. COVID-19 and adult Still’s disease both featured hyperinflammatory status, such as high fever and elevated serum C-reactive protein, whereas COVID-19 and anti-MDA5 dermatomyositis both presented with severe interstitial lung disease and hypoxaemia. While two-thirds of the patients in each group met the criteria for macrophage-activated syndrome that is used in systemic juvenile idiopathic arthritis, the HScore, an indicator of haemophagocytic lymphohistiocytosis, was low in anti-MDA5 dermatomyositis and COVID-19 even in severe or critical cases. The findings of chest computed tomography were similar between COVID-19 and anti-MDA5 dermatomyositis (Figure 1).Conclusion:COVID-19 shared clinical features with rheumatic diseases characterised by hyperferritinaemia, including anti-MDA5 dermatomyositis and adult Still’s disease. These findings should be investigated further in order to shed light on the pathogenesis of not only COVID-19 but also the aforementioned rheumatic diseases.References:[1]Mehta P, McAuley DF, Brown M, et al. COVID-19: consider cytokine storm syndromes and immunosuppression. Lancet. (2020) 395: 1033-4.[2]Gono T, Sato S, Kawaguchi Y, et al. Anti-MDA5 antibody, ferritin and IL-18 are useful for the evaluation of response to treatment in interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis. Rheumatology (Oxford). 2012; 51(9):1563-70.Figure 1.Imaging characteristics of chest CT scans in patients with COVID-19, anti-MDA5 dermatomyositis, and adult Still’s disease A)Bilateral ground-glass and consolidative opacities with peripheral distribution in COVID-19. B)Bilateral ground-glass opacities with peripheral consolidations in anti-MDA5 dermatomyositis. C)Pleural effusion with pleural thickening on the left side in adult Still’s disease.Disclosure of Interests:Yasushi Kondo: None declared., Yuko Kaneko: None declared., Hisoshi Takei: None declared., Hiroya Tamai: None declared., Tsutomu Takeuchi Grant/research support from: received research grants outside the submitted work from Abbvie, Astra Zeneca, Bristol Myers Squibb, Chugai Pharmaceutical, Eisai Pharmaceutical, Janssen Pharmaceutical, Mitsubishi Tanabe Pharma Corporation, Novartis, Takeda Pharmaceutical, Abbott Japan Co., Ltd., Astellas Pharma, Ltd., Daiichi Sankyo, Pfizer, Sanofi–Aventis, Santen Pharmaceutical, Teijin Pharma Ltd., Asahikasei Pharma Corp., SymBio Pharmaceuticals Ltd., Celtrion, Nipponkayaku Co. Ltd., Eli Lilly Japan, and Taisho Toyama Pharmaceutical.

scholarly journals FRI0505 TOCILIZUMAB DISCONTINUATION AFTER REMISSION ACHIEVEMENT IN PATIENTS WITH ADULT STILL’S DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 851.2-851
Author(s):  
H. Tamai ◽  
Y. Kaneko ◽  
T. Takeuchi
Keyword(s):  
Laboratory Data ◽  
Good Control ◽  
Still’S Disease ◽  
Median Dose ◽  
Chugai Pharmaceutical ◽  
Still's Disease ◽  
Adult Still's Disease ◽  
The Mean ◽  
Recurrence Group ◽  
Observation Period

Background:The efficacy of tocilizumab, an interleukin (IL)-6 receptor inhibitor, has been proved in patients with adult Still’s disease on suppressing systemic inflammation and decreasing glucocorticoid dose. However, whether tocilizumab can be discontinued after remission achievement is unclear.Objectives:To clarify the possibility of tocilizumab discontinuation in patients with adult Still’s disease who achieved remission with tocilizumab.Methods:Consecutive patients with adult Still’s disease diagnosed according to the Yamaguchi’s criteria in our hospital from April 2012 until September 2019 were retrospectively reviewed. Patients who were in good control with tocilizumab were included in the analysis, and their clinical courses were collected from their medical charts. Patients were divided according to the presence of recurrence after tocilizumab discontinuation and compared.Results:Among 42 patients with adult Still’s disease who had a history of intravenous tocilizumab of 8mg/kg use, 13 patients discontinued tocilizumab following a good disease control. During the mean observation period of 26.4 months, six patients (46%) remained in remission while seven patients (54%) developed recurrence after tocilizumab discontinuation. The sex and the mean observation period were not different between the patients with recurrence and those without (71% vs 50%, p=0.43; 27.3 months vs 25.4 months, p=0.93, respectively), but the age at tocilizumab discontinuation tended to be higher in the recurrence group than the non-recurrence group (64.0 years vs 46.5 years, p=0.08). The disease activity including swollen joint counts and laboratory data at tocilizumab discontinuation were comparable between the two groups (serum ferritin levels, 88 ng/mL vs 122 ng/mL, p=0.67). While the duration of tocilizumab use was not different between the two groups (29.4 months vs 39.5 months, p=0.40), the mean interval of tocilizumab infusion at tocilizumab discontinuation in the recurrence group was 3.6 weeks, shorter than the 6.7 weeks in the non-recurrence group (p=0.03). The median dose of prednisolone at tocilizumab discontinuation was 5.0 mg/day in the recurrence group and 0.0 mg/day in the non-recurrence group (p=0.06). In the recurrence group, the duration from the last tocilizumab administration to recurrence was 7.8 months, and the median dose of prednisolone at recurrence was 5.0 mg/day.Conclusion:Patients with adult Still’s disease remaining in remission with a longer interval of tocilizumab administration and a lower dose of prednisolone was likely to succeed in withdrawal of tocilizumab.Disclosure of Interests:Hiroya Tamai: None declared, Yuko Kaneko Speakers bureau: Dr. Kaneko reports personal fees from AbbVie, personal fees from Astellas, personal fees from Ayumi, personal fees from Bristol-Myers Squibb, personal fees from Chugai, personal fees from Eisai, personal fees from Eli Lilly, personal fees from Hisamitsu, personal fees from Jansen, personal fees from Kissei, personal fees from Pfizer, personal fees from Sanofi, personal fees from Takeda, personal fees from Tanabe-Mitsubishi, personal fees from UCB, Tsutomu Takeuchi Grant/research support from: Eisai Co., Ltd, Astellas Pharma Inc., AbbVie GK, Asahi Kasei Pharma Corporation, Nippon Kayaku Co., Ltd, Takeda Pharmaceutical Company Ltd, UCB Pharma, Shionogi & Co., Ltd., Mitsubishi-Tanabe Pharma Corp., Daiichi Sankyo Co., Ltd., Chugai Pharmaceutical Co. Ltd., Consultant of: Chugai Pharmaceutical Co Ltd, Astellas Pharma Inc., Eli Lilly Japan KK, Speakers bureau: AbbVie GK, Eisai Co., Ltd, Mitsubishi-Tanabe Pharma Corporation, Chugai Pharmaceutical Co Ltd, Bristol-Myers Squibb Company, AYUMI Pharmaceutical Corp., Eisai Co., Ltd, Daiichi Sankyo Co., Ltd., Gilead Sciences, Inc., Novartis Pharma K.K., Pfizer Japan Inc., Sanofi K.K., Dainippon Sumitomo Co., Ltd.

scholarly journals Association of ocular blood flow and contrast sensitivity in normal tension glaucoma

2021 ◽  
Author(s):  
David Kuerten ◽  
Matthias Fuest ◽  
Peter Walter ◽  
Babac Mazinani ◽  
Niklas Plange
Keyword(s):  
Blood Flow ◽  
Visual Field ◽  
Contrast Sensitivity ◽  
Visual Field Defect ◽  
Prospective Trial ◽  
Normal Tension Glaucoma ◽  
Ocular Blood Flow ◽  
Retinal Blood Flow ◽  
Control Group ◽  
Field Defect

Abstract Purpose To investigate the relationship of ocular blood flow (via arteriovenous passage time, AVP) and contrast sensitivity (CS) in healthy as well as normal tension glaucoma (NTG) subjects. Design Mono-center comparative prospective trial Methods Twenty-five NTG patients without medication and 25 healthy test participants were recruited. AVP as a measure of retinal blood flow was recorded via fluorescein angiography after CS measurement using digital image analysis. Association of AVP and CS at 4 spatial frequencies (3, 6, 12, and 18 cycles per degree, cpd) was explored with correlation analysis. Results Significant differences regarding AVP, visual field defect, intraocular pressure, and CS measurement were recorded in-between the control group and NTG patients. In NTG patients, AVP was significantly correlated to CS at all investigated cpd (3 cpd: r =  − 0.432, p< 0.03; 6 cpd: r =  − 0.629, p< 0.0005; 12 cpd: r =  − 0.535, p< 0.005; and 18 cpd: r =  − 0.58, p< 0.001), whereas no significant correlations were found in the control group. Visual acuity was significantly correlated to CS at 6, 12, and 18 cpd in NTG patients (r =  − 0.68, p< 0.002; r =  − 0.54, p< .02, and r =  − 0.88, p< 0.0001 respectively), however not in healthy control patients. Age, visual field defect MD, and PSD were not significantly correlated to CS in in the NTG group. MD and PSD were significantly correlated to CS at 3 cpd in healthy eyes (r = 0.55, p< 0.02; r =  − 0.47, p< 0.03). Conclusion Retinal blood flow alterations show a relationship with contrast sensitivity loss in NTG patients. This might reflect a disease-related link between retinal blood flow and visual function. This association was not recorded in healthy volunteers.

Adult Still's Disease presenting as PUO; a Not Uncommon Disorder

Rheumatology ◽  
1987 ◽  
Vol 26 (1) ◽  
pp. 65-66 ◽  
Author(s):  
N. COHEN ◽  
J. WEISSGARTEN ◽  
A. GOLIK ◽  
A. PIK ◽  
E. YONA ◽  
...  
Keyword(s):  
Still’S Disease ◽  
Still's Disease ◽  
Adult Still's Disease
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