scholarly journals Orbital pseudotumor: a differential diagnosis of Graves' ophthalmopathy

2011 ◽  
Vol 55 (1) ◽  
pp. 85-88 ◽  
Author(s):  
Luciana Souza Cruz Caminha ◽  
Elisa Rebelo Pinto ◽  
Priscila Alves Medeiros de Sousa ◽  
Ricardo Andrade Oliveira ◽  
Flavia Lucia Conceiçao ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Inflammatory Disease ◽  
Final Diagnosis ◽  
Response To Therapy ◽  
Orbital Pseudotumor ◽  
Graves Ophthalmopathy

The objective of this study is to report and discuss a rare and inflammatory cause of exophthalmos. This report describes a patient with exophthalmos, who was initially diagnosed with euthyroid Graves' with good response to therapy. After 8 years of follow-up, she had recurrence of symptoms and a new evaluation revealed the final diagnosis of orbital pseudotumor. Orbital pseudotumor is an uncommon disorder that both radiologically and clinically mimics a malignant process or other inflammatory disease, such as Graves' ophthalmopathy.

Beneficial effects of intensive plasma exchange followed by immunosuppressive therapy in severe Graves' ophthalmopathy

1986 ◽  
Vol 111 (1) ◽  
pp. 30-38 ◽  
Author(s):  
D. Glinoer ◽  
J. Etienne-Decerf ◽  
M. Schrooyen ◽  
G. Sand ◽  
P. Hoyoux ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Primary Treatment ◽  
Immunosuppressive Drugs ◽  
Response To Therapy ◽  
Ocular Involvement ◽  
Beneficial Effects ◽  
Graves Ophthalmopathy ◽  
Soft Tissue Involvement ◽  
Subsequent Improvement

Abstract. Nine patients with severe Graves' ophthalmopathy were treated by intensive plasma exchange, followed by immunosuppression. Severity of ocular involvement and response to therapy were evaluated clinically by numerical scoring (ophthalmopathy index). Serum thyroid stimulating immunoglobulins (TSI) and urinary excretion of glycosaminoglycans (GAG) were measured immediately before and immediately after plasmapheresis. Plasma exchange was rapidly accompanied by marked clinical improvement in 8/9 patients. The most marked effects were on soft tissue involvement, proptosis, intraocular pressure, and visual acuity. The ophthalmopathy index decreased from 9.7 ± 4.1 to 5.7 ± 2.2 (P <0.001) after plasmapheresis. Serum TSI levels were initially elevated in 6 patients and remained positive in 3 patients after treatment. Urinary GAG excretion was initially 2- to 12-fold normal levels and was decreased by 60%. After plasmapheresis, patients received immunosuppressive drugs for 3–6 months. The follow-up period, after withdrawal of drugs, ranged from 5 to 38 months with a median of 17 months. The ocular condition remained stable in 6 patients. Three patients had a relapse 1 year after plasmapheresis: they were treated a second time by plasma exchange with subsequent improvement. In conclusion, intensive plasma exchange provided prompt and effective improvement in patients with severe progressive Graves' ophthalmopathy. This therapeutic procedure, followed by immunosuppression, gave long lasting results. Relapses were responsive to plasmapheresis therapy. The data suggest that plasma exchange may represent the best primary treatment for severe progressive Graves' ophthalmopathy

scholarly journals ORBITAL PSEUDOTUMOR − CURRENT STATE OF KNOWLEDGE

2020 ◽  
Vol 73 (4) ◽  
pp. 818-822
Author(s):  
Marceli Gacan ◽  
Ewa Papuć ◽  
Konrad Rejdak
Keyword(s):  
Differential Diagnosis ◽  
Literature Review ◽  
Inflammatory Disease ◽  
Clinical Symptoms ◽  
Unknown Origin ◽  
Diagnosis And Treatment ◽  
Orbital Pseudotumor ◽  
Anatomical Structures ◽  
Current State ◽  
Biological Treatments

The orbital pseudo-tumor is an orbital inflammatory disease of unknown origin that can affect all the anatomical structures that make up the orbit. The diagnosis is based on the assessment of clinical symptoms, imaging tests and the exclusion of other possible causes. Glucocorticosteroids are used for treatment, but other immunosuppressants as well as biological treatments can be used. The aim of the study is to present, based on the literature review, the current state of knowledge about pathogenesis, symptoms, differential diagnosis, and treatment of the orbital pseudotumor.

scholarly journals Extraparotid Warthin's tumour: usual tumour adjacent to usual site

1970 ◽  
Vol 10 (4) ◽  
pp. 296-298
Author(s):  
NSA Mutalib ◽  
NM Soleh ◽  
M Irfan
Keyword(s):  
Differential Diagnosis ◽  
Final Diagnosis ◽  
Neck Mass ◽  
Lateral Neck ◽  
Neck Swelling ◽  
Common Presentation ◽  
Warthin’S Tumour ◽  
Usual Site ◽  
Lateral Neck Swelling

Lateral neck swelling is a common presentation in ORL practice. The complexity of the neck structures warrants a thorough examination and investigation to narrow down the differential diagnosis. Neoplasm need to be ruled out especially if the neck swelling present in an adult. We report a case of a lateral neck mass being treated as lymphadenopathy before the patient defaulted follow up. The patient represented after 3 years and investigations performed. FNAC revealed sialadenosis but radiologically and intra-operatively consistently showed the mass located outside parotid gland. The final diagnosis of Whartin's confirmed after the HPE evaluation. The diagnosis dilemma is discussed. Keywords: Lateral neck selling; warthin's tumour; extra parotid DOI: http://dx.doi.org/10.3329/bjms.v10i4.9506 BJMS 2011; 10(4): 296-298

scholarly journals Early recognition of PIMS-TS: a single centre retrospective review

2021 ◽  
Vol 5 (1) ◽  
pp. e001011
Author(s):  
Roshni Mistry ◽  
Nicola Scanlon ◽  
James Hibberd ◽  
Fionnghuala Fuller
Keyword(s):  
Early Recognition ◽  
Febrile Illness ◽  
Final Diagnosis ◽  
University Hospital ◽  
Single Centre ◽  
Retrospective Case ◽  
Reactive Protein ◽  
Review Reports ◽  
Specific Symptoms

IntroductionResearch into paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) has focused on tertiary level management. This review reports on symptoms and investigations at presentation.MethodsSingle centre retrospective case note analysis of patients fulfilling PIMS-TS diagnostic criteria from March to May 2020 in a London district level university hospital.ResultsSix patients presented in the week prior to their final diagnosis with fever and non-specific symptoms. Raised C-reactive protein (CRP), lymphopenia and hyponatraemia were noted. Kawasaki-like symptoms were under-represented in all patients.InterpretationThe results suggest that a proportion of children with early PIMS-TS present with a non-specific febrile illness and abnormal blood results. Further research is needed to determine the most appropriate identification and follow-up of these children.

scholarly journals Primary breast tuberculosis: imaging findings of a rare disease

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Ali H. Baykan ◽  
Hakan S. Sayiner ◽  
Ibrahim Inan ◽  
Elcin Aydin ◽  
Sukru M. Erturk
Keyword(s):  
Differential Diagnosis ◽  
Rare Disease ◽  
Extrapulmonary Tuberculosis ◽  
Reproductive Age ◽  
Malignant Diseases ◽  
Imaging Findings ◽  
Granulomatous Mastitis ◽  
Rare Form ◽  
Breast Tuberculosis

AbstractBreast tuberculosis is a rare form of extrapulmonary tuberculosis which tends to affect females of reproductive age, and is much rarer in males, postmenopausal women, and pre-pubescent girls. It is difficult to diagnose because it can mimic benign conditions such as a fibroadenoma, as well as malignant diseases like a carcinoma. It is also particularly difficult to distinguish breast tuberculosis from granulomatous mastitis, so other possible diagnoses should be ruled out first. The diagnosis of breast tuberculosis has three essential pillars: clinical examination, radiological evaluations, and histopathological sampling. Radiological evaluations are not only critical in diagnosis but are also important in the planning of the treatment and during the follow-up. This paper aims to review the imaging findings and the differential diagnosis of breast tuberculosis.

scholarly journals Rituximab-Containing Risk-Adapted Treatment Strategy in Nodular Lymphocyte Predominant Hodgkin Lymphoma: 7-Years Follow-Up

Cancers ◽  
2021 ◽  
Vol 13 (8) ◽  
pp. 1760
Author(s):  
Novella Pugliese ◽  
Marco Picardi ◽  
Roberta Della Pepa ◽  
Claudia Giordano ◽  
Francesco Muriano ◽  
...  
Keyword(s):  
Side Effects ◽  
Hodgkin Lymphoma ◽  
Prognostic Score ◽  
Single Agent ◽  
Response To Therapy ◽  
Baseline Risk ◽  
Historical Cohort ◽  
Treatment Groups

Background: Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare variant of HL that accounts for 5% of all HL cases. The expression of CD20 on neoplastic lymphocytes provides a suitable target for novel treatments based on Rituximab. Due to its rarity, consolidated and widely accepted treatment guidelines are still lacking for this disease. Methods: Between 1 December 2007 and 28 February 2018, sixteen consecutive newly diagnosed adult patients with NLPHL received Rituximab (induction ± maintenance)-based therapy, according to the baseline risk of German Hodgkin Study Group prognostic score system. The treatment efficacy and safety of the Rituximab-group were compared to those of a historical cohort of 12 patients with NLPHL who received Doxorubicin, Bleomycin, Vinblastine, Dacarbazine (ABVD) chemotherapy followed by radiotherapy (RT), if needed, according to a similar baseline risk. The primary outcome was progression-free survival (PFS) and secondary outcomes were overall survival (OS) and side-effects (according to the Common Terminology Criteria for Adverse Events, v4.03). Results: After a 7-year follow-up (range, 1–11 years), PFS was 100% for patients treated with the Rituximab-containing regimen versus 66% for patients of the historical cohort (p = 0.036). Four patients in the latter group showed insufficient response to therapy. The PFS for early favorable and early unfavorable NLPHLs was similar between treatment groups, while a better PFS was recorded for advanced-stages treated with the Rituximab-containing regimen. The OS was similar for the two treatment groups. Short- and long-term side-effects were more frequently observed in the historical cohort. Grade ≥3 neutropenia was more frequent in the historical cohort compared with the Rituximab-group (58.3% vs. 18.7%, respectively; p = 0.03). Long-term non-hematological toxicities were observed more frequently in the historical cohort. Conclusion: Our results confirm the value of Rituximab in NLPHL therapy and show that Rituximab (single-agent) induction and maintenance in a limited-stage, or Rituximab with ABVD only in the presence of risk factors, give excellent results while sparing cytotoxic agent- and/or RT-related damage. Furthermore, Rituximab inclusion in advanced-stage therapeutic strategy seems to improve PFS compared to conventional chemo-radiotherapy.

scholarly journals Large multicystic spinal lesion in a young African migrant: a problem of differential diagnosis

2021 ◽  
Vol 14 (7) ◽  
pp. e242690
Author(s):  
Tamara Ursini ◽  
Paola Rodari ◽  
Geraldo Badona Monteiro ◽  
Valeria Barresi ◽  
Carmelo Cicciò ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Cystic Echinococcosis ◽  
Bone Cyst ◽  
Spinal Tuberculosis ◽  
Final Diagnosis ◽  
Lower Limbs ◽  
Migration Flow ◽  
Spinal Lesion ◽  
African Migrant ◽  
Expansive Lesion

We describe a rare case of large, fully cystic spinal schwannoma in a young adult from The Gambia. The initial clinical suspicion was spinal cystic echinococcosis. He came to our attention reporting progressive walking impairment and neurological symptoms in the lower limbs. An expansive lesion extending from L2 to S1 was shown by imaging (ie, CT scan and MRI). Differential diagnoses included aneurysmal bone cyst and spinal tuberculosis and abscess; the initial suggested diagnosis of spinal cystic echinococcosis was discarded based on contrast enhancement results. The final diagnosis of cystic schwannoma was obtained by histopathology of the excised mass. Cystic spinal lesions are rare and their differential diagnosis is challenging. Awareness of autochthonous and tropical infectious diseases is important, especially in countries experiencing consistent migration flow; however, it must be kept in mind that migrants may also present with ‘non-tropical’ pathologies.

Excellent Outcome of Acute Necrotizing Encephalopathy in an Adult With Bacterial Infections, Case Report

2021 ◽  
pp. 194187442199137
Author(s):  
Yan Wang ◽  
John R. Younce ◽  
Joel S. Perlmutter ◽  
Soe S. Mar
Keyword(s):  
Differential Diagnosis ◽  
Bacterial Infections ◽  
Vasogenic Edema ◽  
Brain Magnetic Resonance Imaging ◽  
Acute Necrotizing Encephalopathy ◽  
Excellent Outcome ◽  
Intravenous Antibiotics ◽  
Acute Necrotizing ◽  
Supportive Management

Acute necrotizing encephalopathy (ANE) is a rare para-infectious encephalopathy that classically occurs in children. However, ANE should be considered in the differential diagnosis of adults with symmetric brain lesions after a prodromal illness given recent reports of coronavirus disease of 2019 (COVID-19) to presumably cause ANE in adults. We report a case of a 29-year-old male presenting with fever, malaise, and rapid deterioration into coma. Brain magnetic resonance imaging revealed multifocal symmetric areas of diffusion restriction and surrounding vasogenic edema involving bilateral thalami, pons and cerebellar hemispheres with a core of susceptibility artifact, and minimal thalamic contrast enhancement, most consistent with ANE. Extensive infectious workup revealed isolated Escherichia coli and Neisseria gonorrhoeae in his urine. Despite the severe encephalopathy on initial presentation, the patient improved with intravenous antibiotics and supportive management with minimal residual deficits at 9 months follow-up. We aim to provide an overview of the radiological features, differential diagnosis, treatment and prognosis of ANE. Becoming familiarized with this rare but devastating disease will improve detection, treatment, and ultimately prognosis, especially in the era of a new pandemic.

scholarly journals THU0502 EFFICACY AND SAFETY OF SECUKINUMAB TREATMENT IN JUVENILE IDIOPATHIC ARTHRITIS PATIENTS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 489.3-489
Author(s):  
I. Kriulin ◽  
E. Alexeeva ◽  
T. Dvoryakovskaya ◽  
K. Isaeva ◽  
A. Chomakhidze ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Psoriatic Arthritis ◽  
Response To Therapy ◽  
Inactive Disease ◽  
Hla B27 ◽  
Serious Adverse Events ◽  
National Medical ◽  
Alternative Drug ◽  
Baseline Information

Background:Anti-IL-17A biologic drug secukinumab (SEC) proved to be effective for treatment of psoriatic arthritis. However data about its efficacy in juvenile idiopathic arthritis (JIA) are restricted to off-label experience.Objectives:To evaluate the effectiveness and safety of SEC in JIA patients in the National Medical Research Center of Children`s health, Moscow, Russia.Methods:25 patients started SEC therapy from 12/2017 to 11/2019 in single-center prospective study. 3 patients withdrew treatment: two patients (8%) due to AE (1 - allergy followed by MAS after first injection and 1 – leukopenia) and one patient (4%) – after 10 months of treatment due to secondary inefficacy. Among others, 14 patients which were successfully treated for 6 months or longer were included into analysis. At the baseline, information was collected on the characteristics of the onset of the disease, previous therapy and its success. Patients were monitored at least 1 time per year. At each visit, clinical and laboratory characteristics of JIA severity were assessed. Response to therapy was assessed using the ACRPedi 30/50/70/90 criteria, the C.Wallace criteria for inactive disease (WID) and clinical remission. AEs were assessed at each visit.Results:Among 14 patients received SEC for at least 6 months, 7 (50%) have enthesitis-related arthritis, one (7.1%) – persistent oligoarthritis, 4 (28.6%) – RF-negative polyarthritis, 2 (14.3%) – psoriatic arthritis. 6 patients (42.9%) were HLA-B27 positive. Median age of JIA onset was 8.8 (IQR 5:11), age at SEC initiation – 14 (9.9:16.1), disease duration before SEC start – 3.3 (2.7:5.8). 7 (50%) were biologics-naïve, 2 (14.3%) were previously treated with anti-TNF drug, 5 (35.7%) have 2 or more different biologics in anamnesis.SEC demonstrated high efficacy after the first injection resulting in JADAS-71 decreasing in all patients by median 4.3 (1.6:7.1) points and 7/7/5/2 patients (50%/50%/35.7%/14.3%) achieved ACR Pedi 30/50/70/90 response.After 6 months of treatment, WID was achieved by 7 (50%) patients, JADAS-71 decreased from baseline level 15.2 (12.7:20.5) to 0.8 (0:4.2) points, and 14/13/11/9 patients (100%/92.9%/78.6%/64.3%) achieved ACR Pedi 30/50/70/90 response. One patients who had active uveitis at SEC initiation remained with subactive uveitis; one patient with uveitis remission had not flare episodes during follow-up period. One patient (7.1%) had successfully treated evaluation of transaminases after 4-th injection.Conclusion:Secukinumab showed high effectiveness and safety in children with JIA and can be further used both as a first-line drug in JIA associated with HLA-B27, and as an alternative drug for the ineffectiveness of the standard treatment regimen with biologics. No serious adverse events were registered during follow-up period.Disclosure of Interests:Ivan Kriulin: None declared, Ekaterina Alexeeva Grant/research support from: Roche, Pfizer, Centocor, Novartis, Speakers bureau: Roche, Novartis, Pfizer., Tatyana Dvoryakovskaya: None declared, Ksenia Isaeva: None declared, Aleksandra Chomakhidze: None declared, Evgeniya Chistyakova: None declared, Olga Lomakina: None declared, Rina Denisova: None declared, Anna Mamutova: None declared, Anna Fetisova: None declared, Marina Gautier: None declared, Dariya Vankova: None declared, Elizaveta Krekhova: None declared, Meyri Shingarova: None declared, Alina Alshevskaya: None declared, Andrey Moskalev: None declared

scholarly journals POS0941 IN SPONDYLOARTHRITIS PATIENTS THE PRESENCE OF COMORBIDITIES IS AN INDEPENDENT PREDICTOR OF INSUFFICIENT RESPONSE TO THERAPY WITH BIOLOGIC AGENTS AND TREATMENT DISCONTINUATION

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 733.2-734
Author(s):  
I. Flouri ◽  
N. Kougkas ◽  
N. Avgustidis ◽  
A. Repa ◽  
A. Eskitzis ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Disease Duration ◽  
Cox Regression ◽  
Independent Predictor ◽  
Response To Therapy ◽  
Treatment Discontinuation ◽  
Randomized Controlled Studies ◽  
Insufficient Response ◽  
The Impact

Background:Long-term observational studies of patients under biologic disease-modifying anti-rheumatic drug (bDMARD) therapies in routine clinical practice can provide us with important data regarding patients with comorbidities, who are usually excluded from randomized controlled studies.Objectives:To study the impact of comorbidities in the outcome (response and persistence to therapy) of patients with spondyloarthritis (SpA) receiving bDMARDs in real-world clinical practice.Methods:Prospective study of all patients who start a bDMARD in a tertiary centre University Hospital after their consent. All patient comorbidities [among a list of approximately 100 pre-specified major comorbidities] are registered by treating physicians at baseline and during follow-up.Comorbidities were studied as total Comorbidities Count (CC) and rheumatic disease comorbidity index (RDCI). Statistical analyses were performed using logistic and Cox regression models, adjusting for the potential confounding of age, sex, disease duration, diagnosis (axial vs. peripheral SpA), number of previous conventional synthetic and biologic DMARDs, year of therapy start, and co-administered methotrexate and corticosteroids (yes/no). Analyses of response to therapy also included baseline BASDAI or ASDAS indices as confounding variables.Results:A total of 603 biologic treatments (1st: 298, 2nd: 157, ≥3rd: 148) were analyzed. Half (51%) of the patients were female, 413 patients had axial SpA (AxSpA) and 190 peripheral SpA (perSpA). At baseline, median (IQR) age: 48 (38-57) years, disease duration: 11 (4-19) years, CC: 2 (1-4) and RDCI: 1 (0-2). Both comorbidity indices were significantly higher in perSpA compared to AxSpA (p<0.001).At 6 months of therapy, 31% of patients with AxSpA achieved BASDAI50 and 39% had ASDAS-ESR < 2.1. Higher CC was an independent predictor of insufficient response according to BASDAI50 [OR (95%) = 0.70 (0.52-0.94), p=0.019] and higher RDCI was predicting failure to achieve ASDAS-ESR < 2.1 [OR (95%) = 0.59 (0.37-0.94), p=0.027]. Other independent predictors of non-response were age, longer disease duration and (for ASDAS-ESR<2.1) higher baseline disease activity.During 1405 patient-years of follow-up, 349 (58%) treatments were discontinued. The adjusted hazard ratio for bDMARD discontinuation within the first 2 years of treatment due to insufficient response was doubled in patients with CC ≥2 versus those with CC ≤1 [HR = 2.27 (1.14-4.53), p=0.020] or with RDCI ≥1 (vs. RDCI = 0) [HR = 2.23 (1.22-4.07), p=0.009]. Comorbidities’ indices were not significant predictors of treatment discontinuations due to adverse events.Conclusion:The presence of comorbidities in patients with SpA is an independent predictor for insufficient 6-month response to bDMARDs and resultant treatment discontinuation due to failure.Acknowledgements:This research is co-financed by Greece and the European Union (European Social Fund- ESF) through the Operational Programme «Human Resources Development, Education and Lifelong Learning» in the context of the project “Reinforcement of Postdoctoral Researchers - 2nd Cycle” (MIS-5033021), implemented by the State Scholarships Foundation (ΙΚΥ).Disclosure of Interests:None declared

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