scholarly journals IgG4-Related Ophthalmic Disease Presenting as Meningitis and Panuveitis

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Maria A. Mavrommatis ◽  
Sarah A. Avila ◽  
Richard France

Purpose. We report an uncommon case of immunoglobulin gamma 4-related ophthalmic disease (IgG4-ROD) presenting as meningitis and panuveitis. Observations. A 35-year-old male with no prior ophthalmic history presented with headaches, altered mental status, and fever of unknown origin. A lumbar puncture (LP) revealed an elevated white count with lymphocytic predominance, confirming a suspected meningitis. After an extensive work-up, he was discharged on oral acyclovir to cover for presumed aseptic meningitis. The patient initially improved, however, bilateral eye pain, redness, and photophobia 2 weeks after discharge prompted his first visit to the ophthalmology clinic. Exam at that time was consistent with bilateral anterior uveitis for which he was given topical prednisolone and cyclopentolate. In addition to the preceding work-up, quantitative immunoglobulin serology including IgG4 levels was added. At follow-up, he was found to have increased ocular inflammation with vitreitis, nerve head edema, and subclinical macular thickening. Visual acuity (VA) had decreased in both eyes. Serology titers for IgG had resulted in a significant elevation in IgG subclass 4 (IgG4). Optical coherence tomography (OCT) and fundus fluorescein angiography (FFA) confirmed posterior retinal involvement. The patient was diagnosed with presumed bilateral panuveitis secondary to IgG4-ROD. Conclusions and Importance. IgG4-RD can be a serious condition that requires careful consideration and intuition to diagnose. This report serves to encourage ophthalmologists to consider IgG4-ROD in cases of idiopathic systemic inflammation with ophthalmic involvement.

2019 ◽  
Vol 33 (02) ◽  
pp. 103-105 ◽  
Author(s):  
Mofiyinfolu Sokoya ◽  
Jason Cohn ◽  
Scott Kohlert ◽  
Thomas Lee ◽  
Sameep Kadakia ◽  
...  

AbstractOrbital exenteration (OE) is a radical operation associated with significant psychosocial disability and functional impairment. Indications for OE include primary tumors of the eye, oral cavity, paranasal sinuses, skin, and brain. Careful consideration regarding the likelihood of local control and cure is needed before proceeding with this operation. Multidisciplinary work-up should be performed before proceeding with surgery. The method of reconstruction after OE should be tailored to the defect and the postoperative needs of the patient. Appropriate follow-up and rehabilitation should be arranged for the patient.


2004 ◽  
Vol 43 (05) ◽  
pp. 171-176 ◽  
Author(s):  
T. Behr ◽  
F. Grünwald ◽  
W. H. Knapp ◽  
L. Trümper ◽  
C. von Schilling ◽  
...  

Summary:This guideline is a prerequisite for the quality management in the treatment of non-Hodgkin-lymphomas using radioimmunotherapy. It is based on an interdisciplinary consensus and contains background information and definitions as well as specified indications and detailed contraindications of treatment. Essential topics are the requirements for institutions performing the therapy. For instance, presence of an expert for medical physics, intense cooperation with all colleagues committed to treatment of lymphomas, and a certificate of instruction in radiochemical labelling and quality control are required. Furthermore, it is specified which patient data have to be available prior to performance of therapy and how the treatment has to be carried out technically. Here, quality control and documentation of labelling are of greatest importance. After treatment, clinical quality control is mandatory (work-up of therapy data and follow-up of patients). Essential elements of follow-up are specified in detail. The complete treatment inclusive after-care has to be realised in close cooperation with those colleagues (haematology-oncology) who propose, in general, radioimmunotherapy under consideration of the development of the disease.


2014 ◽  
Vol 23 (2) ◽  
pp. 179-185 ◽  
Author(s):  
Suvadip Chatterjee ◽  
Kofi W. Oppong ◽  
John S. Scott ◽  
Dave E. Jones ◽  
Richard M. Charnley ◽  
...  

Background & Aims: Autoimmune pancreatitis (AIP) is a fibroinflammatory condition affecting the pancreas and could present as a multisystem disorder. Diagnosis and management can pose a diagnostic challenge in certain groups of patients. We report our experience of managing this condition in a tertiary pancreaticobiliary centre in the North East of England.Methods: Patients were identified from a prospectively maintained database of patients diagnosed with AIP between 2005 and 2013. Diagnosis of definite/probable AIP was based on the revised HISORt criteria. When indicated, patients were treated with steroids and relapses were treated with azathioprine. All patients have been followed up to date.Results: Twenty-two patients were diagnosed with AIP during this period. All patients had pancreatic protocol CT performed while some patients had either MR or EUS as part of the work up. Fourteen out of 22 (64%) had an elevated IgG4 level (mean: 10.9 g/L; range 3.4 - 31 g/L). Four (18%) patients underwent surgery. Extrapancreatic involvement was seen in 15 (68%) patients, with biliary involvement being the commonest. Nineteen (86%) were treated with steroids and five (23%) required further immunosuppression for treatment of relapses. The mean follow up period was 36.94 months (range 7 - 94).Conclusion: Autoimmune pancreatitis is being increasingly recognized in the British population. Extrapancreatic involvement, particularly extrahepatic biliary involvement seems to be a frequent feature.Diagnosis should be based on accepted criteria as this significantly reduces the chances of overlooking malignancy. Awareness of this relatively rare condition and a multi-disciplinary team approach will help us to diagnose and treat this condition more efiectively thereby reducing unnecessary interventions.


1970 ◽  
Vol 5 (4) ◽  
pp. 61-66
Author(s):  
Patrícia Resende Penido ◽  
Rhanna Junqueira Westin de Carvalho ◽  
Roger Willian Moraes Mendes

RESUMOIntrodução: A Arterite de Takayasu (AT) consiste em uma vasculopatia de origem indefinida, sendo de caráter crônico, que afeta a aorta e seus ramos principais. Em gestantes é uma condição complexa, em que o tratamento clínico é realizado com restrições. A utilização de corticoides tem sido favorável no controle inflamatório, principalmente naqueles casos em que antes da gravidez se fazia uso de imunossupressores. Casuística: Foi relatado um caso de uma gestante portadora de AT, através da análise de prontuário e de exames complementares, sendo realizado o pré-natal pelas equipes de Obstetrícia e Reumatologia, onde foi realizado tratamento clínico com corticoides, mostrando uma evolução satisfatória, ocorrendo apenas uma hospitalização que foi seguida de uma cesárea na trigésima sexta semana de gravidez, com recém nato saudável de 3.810g. Discussão: A AT pode estar associada a várias etiologias, sendo a gênese pouco conhecida. O diagnóstico na maioria das vezes é demorado, pela dificuldade da suspeita clínica, além de demandar o uso de técnicas de imagem mais sofisticadas. A gestação associada é fenômeno raro, já que as portadoras são orientadas a evitarem a gravidez. O controle clínico permitiu uma gestação sem grandes complicações e serviu como meio para levar a gestação até praticamente o fim do terceiro trimestre. Conclusão: Deve-se ressaltar o papel do acompanhamento clínico, especialmente com esta pan-arterite, mostrando os medicamentos que podem ser utilizados nesse grupo, especialmente quando se usava imunossupressores antes da gravidez. Palavras chave: Arterite de Takayasu, Gravidez, Vasculite. ABSTRACT:Introduction: Takayasu's arteritis (TA) consists of a vascular disease of unknown origin and chronic nature, which affects the aorta and its main branches. In pregnant women it is a complex condition, in which the clinical treatment is performed with restrictions. The use of corticosteroids has been favorable to control inflammation, especially in those cases when immunosuppressant had been used before pregnancy. Case Report: A case of a pregnant patient with TA has been reported through the analysis of medical records and laboratory tests. The Obstetrics and Rheumatology staff performed the prenatal care, and clinical treatment with corticosteroids was done showing a satisfactory outcome. There was only one hospitalization of the patient, which was followed by a cesarean in the thirty sixth week of pregnancy.  The newborn was a healthy one, weighing 3.810g. Discussion: TA may be associated with several etiologies, and the genesis is little known. The diagnosis most often takes time due to the difficulty of clinical suspicion, and requires the use of more sophisticated imaging techniques. The pregnancy is rare phenomenon, since the carriers are advised to avoid pregnancy. The clinical management allowed a pregnancy without major complications and served as a means to carry the pregnancy to almost the end of the third quarter. Conclusion: The role of the clinical follow-up should be noticed especially with this pan-arteritis, showing the drugs that can be used in this group, especially when there is use of immunosuppressive drugs before pregnancy. Keywords: Takayasu Arteritis, Pregnancy, Vasculitis.


Author(s):  
C. J. Koppel ◽  
B. W. Driesen ◽  
R. J. de Winter ◽  
A. E. van den Bosch ◽  
R. van Kimmenade ◽  
...  

Abstract Background Current guidelines on coronary anomalies are primarily based on expert consensus and a limited number of trials. A gold standard for diagnosis and a consensus on the treatment strategy in this patient group are lacking, especially for patients with an anomalous origin of a coronary artery from the opposite sinus of Valsalva (ACAOS) with an interarterial course. Aim To provide evidence-substantiated recommendations for diagnostic work-up, treatment and follow-up of patients with anomalous coronary arteries. Methods A clinical care pathway for patients with ACAOS was established by six Dutch centres. Prospectively included patients undergo work-up according to protocol using computed tomography (CT) angiography, ischaemia detection, echocardiography and coronary angiography with intracoronary measurements to assess anatomical and physiological characteristics of the ACAOS. Surgical and functional follow-up results are evaluated by CT angiography, ischaemia detection and a quality-of-life questionnaire. Patient inclusion for the first multicentre study on coronary anomalies in the Netherlands started in 2020 and will continue for at least 3 years with a minimum of 2 years of follow-up. For patients with a right or left coronary artery originating from the pulmonary artery and coronary arteriovenous fistulas a registry is maintained. Results Primary outcomes are: (cardiac) death, myocardial ischaemia attributable to the ACAOS, re-intervention after surgery and intervention after initially conservative treatment. The influence of work-up examinations on treatment choice is also evaluated. Conclusions Structural evidence for the appropriate management of patients with coronary anomalies, especially (interarterial) ACAOS, is lacking. By means of a structured care pathway in a multicentre setting, we aim to provide an evidence-based strategy for the diagnostic evaluation and treatment of this patient group.


2021 ◽  
Vol 11 (3) ◽  
pp. 178
Author(s):  
Noah R. Delapaz ◽  
William K. Hor ◽  
Michael Gilbert ◽  
Andrew D. La ◽  
Feiran Liang ◽  
...  

Post-traumatic stress disorder (PTSD) is a prevalent mental disorder marked by psychological and behavioral changes. Currently, there is no consensus of preferred antipsychotics to be used for the treatment of PTSD. We aim to discover whether certain antipsychotics have decreased suicide risk in the PTSD population, as these patients may be at higher risk. A total of 38,807 patients were identified with a diagnosis of PTSD through the ICD9 or ICD10 codes from January 2004 to October 2019. An emulation of randomized clinical trials was conducted to compare the outcomes of suicide-related events (SREs) among PTSD patients who ever used one of eight individual antipsychotics after the diagnosis of PTSD. Exclusion criteria included patients with a history of SREs and a previous history of antipsychotic use within one year before enrollment. Eligible individuals were assigned to a treatment group according to the antipsychotic initiated and followed until stopping current treatment, switching to another same class of drugs, death, or loss to follow up. The primary outcome was to identify the frequency of SREs associated with each antipsychotic. SREs were defined as ideation, attempts, and death by suicide. Pooled logistic regression methods with the Firth option were conducted to compare two drugs for their outcomes using SAS version 9.4 (SAS Institute, Cary, NC, USA). The results were adjusted for baseline characteristics and post-baseline, time-varying confounders. A total of 5294 patients were eligible for enrollment with an average follow up of 7.86 months. A total of 157 SREs were recorded throughout this study. Lurasidone showed a statistically significant decrease in SREs when compared head to head to almost all the other antipsychotics: aripiprazole, haloperidol, olanzapine, quetiapine, risperidone, and ziprasidone (p < 0.0001 and false discovery rate-adjusted p value < 0.0004). In addition, olanzapine was associated with higher SREs than quetiapine and risperidone, and ziprasidone was associated with higher SREs than risperidone. The results of this study suggest that certain antipsychotics may put individuals within the PTSD population at an increased risk of SREs, and that careful consideration may need to be taken when prescribed.


2021 ◽  
Vol 2 (1) ◽  
pp. 75-86
Author(s):  
Maria Irene Bellini ◽  
Vito Cantisani ◽  
Augusto Lauro ◽  
Vito D’Andrea

Living kidney donation represents the best treatment for end stage renal disease patients, with the potentiality to pre-emptively address kidney failure and significantly expand the organ pool. Unfortunately, there is still limited knowledge about this underutilized resource. The present review aims to describe the general principles for the establishment, organization, and oversight of a successful living kidney transplantation program, highlighting recommendation for good practice and the work up of donor selection, in view of potential short- and long-terms risks, as well as the additional value of kidney paired exchange programs. The need for donor registries is also discussed, as well as the importance of lifelong follow up.


2003 ◽  
Vol 44 (4) ◽  
pp. 444-446
Author(s):  
R. Dullerud ◽  
A. Server ◽  
J. Berg-Johnsen

We report on 2 patients in whom a cystic dilation of the conus medullaris was incidentally found at MR imaging carried out in the work-up for sciatica. The cysts were well circumscribed and had signal intensity identical to the CSF on both T1- and T2-weighted images. There was no evidence of contrast enhancement. None of the patients had specific symptoms related to the spinal cord. At surgery, no evidence of malignancy was seen in any of the patients. A benign cystic dilation, also called dilated ventriculus terminalis, occasionally can be seen in the conus medullaris as an incidental finding at thoracolumbar MR imaging. Unless the expansion per se indicates cyst drainage, these patients may be monitored by clinical and MR follow-up, avoiding surgery in a substantial number of cases.


PEDIATRICS ◽  
1989 ◽  
Vol 83 (6) ◽  
pp. 986-992
Author(s):  
Yardena Rakover ◽  
Hanna Adar ◽  
Itamar Tal ◽  
Yaron Lang ◽  
Amos Kedar

Behcet disease is rare in children. There are only two reports of Behcet disease in childhood, describing seven patients. Three pediatric patients are described, in whom the age of onset ranged from 6 to 11 years. Aphthous stomatitis and arthritis were present in all of the patients; genital ulcers, iridocylitis, erythema nodosum, and CNS involvement were present in two patients. Other manifestations included Stevens-Johnson-like eruption, fever of unknown origin, and testicular involvement. All of the patients responded to glucocorticoids; two were also treated with colchicine and one was treated with chlorambucil. In two patients, follow-up of more than 10 years was done, with complete cure in one patient and benign course of illness in the other. Because of the rarity of the disease in childhood and the difficulty in making the diagnosis, there is not enough awareness by pediatricians concerning this disease.


2021 ◽  
Vol 4 (5) ◽  
pp. 01-04
Author(s):  
Salma Fares ◽  
Adil Taoufik ◽  
Aissam Maataoui ◽  
Kaoutar Sokori ◽  
Ouadie Qamouss

Background: Solitary plasmacytoma is a rare malignant neoplasm of plasma cells accounting 5-10% of all plasma cell dyscrasias with extramedullary plasmocytoma in 3- 5%. Their localization in the female genital tract is quite rare, either as solitary plasmacytomas or as part of a disseminated MM. Solitary ovarian plasmocytoma is extremely rare. Case: A 52-year-old woman, presented presented postmenopausal recurring episodes of metrorrhagia with left ovarian mass. She was diagnosed with solitary ovarianplasmocytoma without systemicdisease. The patient underwent complete surgery resection and a full work up to rule out multiple myeloma that objectived a small serum monoclonal protein that had resolved postoperatively. At 17 months of follow-up, the patient is still alive and doing well with no signs of recurrence or progression to mutliple myeloma. Although rare, solitary plasmacytoma of the ovary can occur without any overt symptoms or laboratory abnormalities tests and require prompt and adequate treatment and rigorous monitoring due to their ability to relapse or progress to MM. Complete surgical resection followed by activesurveillance is appropriate.


Sign in / Sign up

Export Citation Format

Share Document