Case of vaso-occlusive retinopathy in Kikuchi-Fujimoto and lupus overlap syndrome

2021 ◽  
Vol 14 (5) ◽  
pp. e240752
Author(s):  
Nima Ghadiri ◽  
Miles Stanford
Keyword(s):  
Lupus Erythematosus ◽  
Disease Process ◽  
Lymph Node Biopsy ◽  
Overlap Syndrome ◽  
Benign Condition ◽  
Appropriate Treatment ◽  
Retinal Microvasculature ◽  
Systemic Symptoms ◽  
Sequential Reduction

A 35-year-old woman presented with a constellation of systemic symptoms: rashes, weight loss, arthralgia and mouth ulcers. Six months afterwards, she experienced bilateral and sequential reduction in vision, and was found to have bilateral vaso-occlusive retinopathy, with critical macular ischaemia in the left eye. Her serological markers were consistent with a diagnosis of lupus. A lymph node biopsy confirmed Kikuchi-Fujimoto disease, a benign condition of unknown cause characterised by fever, cervical and axillary lymphadenopathy. Given that this overlap syndrome was associated with a number of systemic features and had affected the eyes, an immunosuppressive regime with rituximab was considered prudent. This rendered her vasculitis stable and non-progressive, and there were signs of partial retinal microvasculature recovery on optical coherence tomography angiography. There is increasing evidence of an overlap between Kikuchi-Fujimoto disease and systemic lupus erythematosus, which is associated with vaso-occlusive retinopathy. In these instances, a multidisciplinary approach is warranted, with consideration of appropriate treatment in order to prevent harmful sequelae of vasculitis. Our treatment with rituximab abated the disease process, although close follow-up is paramount to monitor results and side-effects of treatment.

A case of upper extremity deep vein thrombosis due to Kikuchi Fujimoto disease

VASA ◽  
2012 ◽  
Vol 41 (5) ◽  
pp. 371-374
Author(s):  
Belaj ◽  
Gary ◽  
Eller ◽  
Deutschmann ◽  
Beham-Schmid ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Upper Extremity ◽  
Lupus Erythematosus ◽  
Lymph Node Biopsy ◽  
Vein Thrombosis ◽  
Node Biopsy ◽  
Necrotizing Lymphadenitis ◽  
Systemic Symptoms ◽  
Deep Vein

Kikuchi Fujimoto disease (KFD) is a rare form of lymphadeopathy with systemic symptoms. We present a case of a 31-year-old female farmer who was admitted to the emergency ward because of swelling of the left arm. Upper extremity venous thrombosis due to local compression of lymph nodes was diagnosed. The histological workup of lymph node biopsy showed histiocytic necrotizing lymphadenitis which is a typical sign of KFD. Anticoagulant treatment for the venous thrombosis was initiated. As KFD is often associated with a transition to systemic lupus erythematosus follow up visits are scheduled in our outpatient clinic.

Ayurvedic Management of Systemic Lupus Erythematosus Overlap Vasculitis vis a vis Vatarakta - A Case Report

2018 ◽  
Vol 3 (5) ◽  
Author(s):  
Dr. Gururaja D. ◽  
Dr. Veeraj Hegde
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Lupus Erythematosus ◽  
Severe Pain ◽  
Overlap Syndrome ◽  
Burning Sensation ◽  
Tinospora Cordifolia ◽  
Systemic Lupus ◽  
Multisystem Disease

Systemic Lupus erythematosus is the classic prototype of multisystem disease of autoimmune origin. SLE may be associated Vasculitis as an overlap syndrome. In this paper, a patient diagnosed as SLE with Vasculitis, which was managed successfully by Ayurveda treatment is discussed. A 39 year old female patient came to hospital with complaint of severe pain and burning sensation in both the legs for two months, associated with ulceration and gangrene of toes of both the legs for the last 15 days. She was diagnosed as SLE overlap vasculitis at a higher medical centre with relevant investigations and advised to go for amputation. As patient was not willing for surgery came to Ayurveda treatment. The condition was diagnosed as disease Vatarakta and treatment was planned accordingly. Treatment was planned by selecting suitable oral medicines, planning suitable Panchakarma procedures along with the ulcer management. Guduchi (Tinospora cordifolia) was the main drug which is used in Rasayana dosage. Patient responded well and we could able to save the limb. Patient was under follow up for more than a year without any complications and relapses.

scholarly journals The Various Forms of Nephrotic Syndrome in a Patient with Systemic Lupus Erythematosus

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Sophia Lionaki ◽  
George Liapis ◽  
Kalliope Vallianou ◽  
Chrysovalantis Vergadis ◽  
Ioannis Boletis
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Nephrotic Syndrome ◽  
Lupus Erythematosus ◽  
Disease Onset ◽  
Systemic Lupus ◽  
Kidney Involvement ◽  
Lupus Podocytopathy ◽  
Systemic Symptoms ◽  
Serological Activity

Kidney involvement is frequent in patients with systemic lupus erythematosus (SLE), although it may not be present from disease onset. Renal lupus itself is highly heterogenous with respect to the combination and/or severity of clinical and/or laboratory manifestations. This is a case of a 45-year-old Caucasian female with an established diagnosis of SLE, who presented four times with new onset of proteinuria during a follow-up time of ten years, since the diagnosis of SLE. Specifically, she experienced two episodes of lupus membranous nephropathy, and after she achieved remission, she developed twice overt nephrotic syndrome associated with new and biopsy proven lupus podocytopathy. All these episodes of nephrotic syndrome were combined with systemic symptoms, attributed to lupus itself, while serological activity of lupus was also noted. This case highlights the importance of performing a kidney biopsy in all patients with SLE who have new renal manifestations, including nephrotic proteinuria.

scholarly journals A CASE OF RECURRENT MISCARRIAGES WITH ANAEMIA

2020 ◽  
pp. 28-29
Author(s):  
Kolluru V D Karthik ◽  
Valeti Rajeswari
Keyword(s):  
Past History ◽  
Disease Process ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Young Females ◽  
Pregnancy Morbidity ◽  
Appropriate Treatment ◽  
History Of ◽  
Auto Antibody

APLAS/ APS is an auto antibody mediated acquired thrombophilia with recurrent arterial / venous thrombosis and pregnancy morbidity . It primarily affects females . 5 cases per 1,00,000 population Diagnosis of APLAS should be considered in cases of thrombosis, CVA in individuals less than 55yrs of age . We report a case of a 21 yr old female who had history of jaundice ( on and off episodes) excessive menstrual bleeding with past history of 3 miscarriages. She was screened and evaluated for Autoimmune diseases and found to have ANTIPHOSPHOLIPID ANTIBODY SYNDROME . She was kept on anticoagulants and aspirin . And patient was doing well on follow up. Any miscarriages/ anaemia in young females needs active investigation so that appropriate treatment can be started to halt the disease process.

scholarly journals AB0593 DOES REALLY EXIST MIXED CONNECTIVE TISSUE DISEASE?

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1593.1-1593
Author(s):  
L. Montolio-Chiva ◽  
J. Narváez ◽  
M. Pascual ◽  
H. S. Park ◽  
A. V. Orenes Vera ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Mixed Connective Tissue Disease ◽  
Overlap Syndrome ◽  
Average Score ◽  
Systemic Autoimmune Disease ◽  
Independent Entity

Background:Currently, most authors accept that mixed connective tissue disease (MCTD) is an independent entity, although there are those who argue that it is actually an overlap syndrome or an undifferentiated early phase of another systemic autoimmune disease (SAD).Objectives:To analyze the long term evolution of a serie of patients with MCTD.Methods:Observational, retrospective and multicenter study in patients with MCTD (diagnostic criteria of Alarcón-Segovia et al),followed for a minimun of 2 years.Results:Fifty-five patients (49 women) with a median age at diagnosis of 38±14 years and with a follow up time (median) of 101 months (range, 24-237 months with a total of 501.2 pacients-year) were identified.At the end of the follow-up period, only 27% (15/55) of the patients kept on fulfilling MCTD criteria. In the remaining 73% (40), 40% (22) had been differentiated to systemic lupus erythematosus (SLE), 13% (7) to systemic sclerosis (SSc) and 20% (11) developed an overlap syndrome [SSc+SLE in 8 cases and SSc+rheumatoid arthritis (AR) in 3]. In 8% of these patients, a secondary Sjögren’s syndrome was diagnosed during the follow-up period. The average score in patients who met the EULAR/ACR 2013 criteria for SSc was 11 (minimum 9 - maximum 16) and the average time elapsed from the diagnosis of MCTD to meet SSc criteria was 64.4 months (interquartile range [IQR] 25-75%: 10-127 months).Applying the 2012 SLICC criteria, only 24 patients of those initially diagnosed as MCTD ended up meeting SLE criteria. The average score in these patients was 5.6 (4-9) and the average time elapsed from the diagnosis of MCTD unltil fulfilling the SLICC criteria was 39 months (IQR 25-75%: 6-28). When we apply the new ACR/EULAR 2019 criteria, the percentage of patients who meet SLE criteria increased to 30%, with an average score of 17.3 (10-38). The average time elapsed since the diagnosis of MCTD until meeting the new SLE criteria was reduced to 17 months (IQR 25-75: 0-10).In the multivariate study, the presence of sclerodactyly (OR: 2.91; IC 95% 1.90 - 4.1, p= 0.001) and esophageal involvement (OR: 2.05; IC 95% 1.14–3.66, p=0.016) were associated with the evolution to SSc. Any predictor of evolution to SLE was identified.Conclusion:Only slightly more than a quarter of patients initially diagnosed as MCTD maintain this diagnosis during the follow-up. The majority, ended up evolving towards to another SAD, fundamentally SLE and SSc. The new ACR/EULAR 2019 criteria seems to be more sensitive than the SLICC 2012 criteria for diagnose SLE in these patients.Disclosure of Interests:L Montolio-Chiva: None declared, J. Narváez: None declared, Maria Pascual: None declared, Hye Sang Park: None declared, Ana V Orenes Vera: None declared, Eduardo Flores: None declared, Juanjo J Alegre-Sancho Consultant of: UCB, Roche, Sanofi, Boehringer, Celltrion, Paid instructor for: GSK, Speakers bureau: MSD, GSK, Lilly, Sanofi, Roche, UCB, Actelion, Pfizer, Abbvie, Novartis, Iván Castellví: None declared, Joan Miquel Nolla: None declared

scholarly journals Kikuchi Fujimoto as an Initial Presentation of Systemic Lupus Erythromatosis

2021 ◽  
Vol 41 (1) ◽  
pp. 99-102
Author(s):  
Srijana Basnet ◽  
Laxman Shrestha ◽  
Prabina Shrestha
Keyword(s):  
Case Report ◽  
Lymph Node ◽  
Axillary Lymph Node ◽  
Lymph Node Biopsy ◽  
Axillary Lymph ◽  
Systemic Lupus ◽  
Benign Condition ◽  
Node Biopsy ◽  
Common Causes

Kikuchi-Fujimoto Disease (KFD) is a rare benign, condition of necrotising histiocytic lymphadenitis. In this case report, we discuss a case of 10 year old male patient who presented with a fever, rash and generalised lymphadenopathy that was not attributable to the more common causes. Axillary lymph node biopsy confirmed the diagnosis of KFD. Treatment with prednisolone improved his symptoms but after six months he had recurrence of his symptoms. He was investigated again and finally met diagnostic criteria for SLE. This case report highlights importance of close follow up in a child with KFD.

When fever is more than infection: two cases of vancomycin-induced drug reaction with eosinophilia and systemic symptoms (DRESS)

2021 ◽  
Vol 14 (1) ◽  
pp. e238006
Author(s):  
Mitchell Cox ◽  
Sophie Paviour ◽  
Sophie Gregory ◽  
Rusheng Chew
Keyword(s):  
Drug Reaction ◽  
Cytomegalovirus Reactivation ◽  
First Case ◽  
Metropolitan Hospital ◽  
Orthopaedic Ward ◽  
Systemic Hypersensitivity ◽  
Systemic Symptoms ◽  
Inflammatory Syndrome ◽  
Intravenous Glucocorticoids

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, but serious systemic hypersensitivity reaction associated with a range of medications. We present two cases of vancomycin-induced DRESS, which occurred simultaneously in the orthopaedic ward in an outer metropolitan hospital. These cases demonstrate the complexity in the diagnosis and management of this inflammatory syndrome on the background of known infection as well as evidence for linezolid as an alternative to vancomycin. The first case was managed conservatively, but developed progressive renal and liver injury along with demonstrated cytomegalovirus reactivation and recurrent colitis, and was eventually palliated. The second was commenced on intravenous glucocorticoids and achieved remission, although had ongoing renal dysfunction at the time of discharge from outpatient follow-up.

scholarly journals POS0204 AUTOANTIBODIES AND SYSTEMIC LUPUS ERYTHEMATOSUS INDUCED BY ANTI-TUMOUR NECROSIS FACTOR ALPHA THERAPY IN RHEUMATOID ARTHRITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 318.1-318
Author(s):  
D. Santos Oliveira ◽  
A. Martins ◽  
F. R. Martins ◽  
F. Oliveira Pinheiro ◽  
M. Rato ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Seroconversion Rate ◽  
Necrosis Factor Alpha ◽  
Malar Rash ◽  
Tnf Α ◽  
Factor Alpha ◽  
Necrosis Factor ◽  
Over Time

Background:Anti-tumour necrosis factor alpha (anti-TNF-α) therapy is commonly used to treat inflammatory conditions such as rheumatoid arthritis (RA). Autoantibodies namely antinuclear antibodies (ANA) induced by these treatments are well established. However, anti-TNF-α-induced systemic lupus erythematosus (SLE) is rarely described and its incidence is yet unknown.Objectives:This study aimed to determine the prevalence of ANA seroconversion and to characterize the development of SLE induced by anti-TNF-α therapy in patients with RA over time.Methods:An observational retrospective cohort study was conducted with at least one year of follow-up. Patients with diagnosis of RA, according to American College of Rheumatology criteria (ACR), and registered on Rheumatic Diseases Portuguese Register (Reuma.pt) who started their first anti-TNFα between 2003 and 2019 were included. Patients with positive ANA (titer ≥100) and/or positive double-strand DNA (dsDNA) antibodies and/or with a diagnosis of SLE at their first visit were excluded. Demographic, clinical and laboratory data were obtained by consulting Reuma.pt. As there are no recognized criteria for drug-induced SLE, the diagnosis of SLE induced by anti-TNF-α was considered if there is a temporal relationship between clinical manifestations and anti-TNF-α-therapy, the presence of at least 1 serologic ACR criteria (ANA or anti-dsDNA) and at least 1 nonserologic ACR criteria (arthritis, serositis, hematologic disorder or malar rash) [1]. Continuous variables are presented with mean, standard deviation, median, quartile 1 and quartile 3. Categorical variables are presented with absolute and relative frequencies.Results:A total of 211 patients (mean age of 49.9±10.9 years old; 84.4% female) were included with a median follow-up time of 6 [3-14] years. We found a seroconversion rate for ANA of 75.4% (n=159) with median treatment duration of 31 [8.5-70.5] months. The most common titre was 1/100 with diffuse and speckled patterns. ANA seroconversion was higher for etanercept (47.8%, n=76) than with adalimumab (23.9%, n=38), infliximab (13.8%, n=22), golimumab (12.6%, n=20) or certolizumab (1.9%, n=3). SLE induced by anti-TNF-α occurred in two patients (0.9%) with erosive and seropositive (rheumatoid factor and anti-citrullinated protein antibodies) RA previously treated with two conventional synthetic disease-modifying antirheumatic drugs, including methotrexate. The first patient, a female with 66 years old and 17 years of disease duration, developed SLE after 16 months of infliximab, with constitutional symptoms, abrupt worsening of polyarthritis, ANA titer of 1/320 diffuse pattern and positive dsDNA (248 UI/mL) antibodies. The second patient, a woman with 43 years old and 11 years of disease duration, developed SLE after 41 months of adalimumab with malar rash and ANA titer of 1/320 diffuse pattern, positive dsDNA (285 UI/mL), positive anti-histone antibodies and hypocomplementemia. In these two cases, anti-TNF-α therapy was stopped and recovery was spontaneous without treatment. The first patient switched to adalimumab and the second switched to golimumab without recurrence of SLE for more than ten years.Conclusion:We found a high rate of ANA seroconversion induced by anti-TNFα therapy in patients with RA. However, similar to previous literature, only 0.9% of patients developed SLE with mild manifestations without major organ involvement. Although the drug with the highest ANA seroconversion rate was etanercept, those responsible for induced SLE were infliximab and adalimumab. Patients improved after discontinuation of therapy and tolerated an alternative anti-TNF-α drug without recurrence of induced SLE over time. Therefore, ANA and SLE induced by anti-TNF-α should be considered and reported in the follow-up of RA patients. Further research is needed to explore the impact of this adverse event on the outcomes of treatment over time.References:[1]Hochberg MC. Arthritis Rheum. 1997;40(9):1725.Disclosure of Interests:None declared

scholarly journals POS0693 EFFICACY AND SAFETY OF BELIMUMAB IN PATIENTS WITH LUPUS NEPHRITIS IN REAL-LIFE SETTING: RESULTS FROM A LARGE, NATIONWIDE, MULTICENTRIC, PROSPECTIVE COHORT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 594-595
Author(s):  
F. Saccon ◽  
M. Gatto ◽  
M. Zen ◽  
M. Fredi ◽  
F. Regola ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Independent Predictor ◽  
Rescue Therapy ◽  
Real Life ◽  
Multivariate Logistic Regression Analysis ◽  
Class Iii ◽  
Baseline Serum ◽  
Renal Response ◽  
Real Life Setting

Background:LN is still a severe manifestation of Systemic lupus erythematosus (SLE) and multitarget therapy is needed to control the disease especially in refractory cases.Objectives:To evaluate renal response in SLE patients with glomerulonephritis (GN) treated with Belimumab in real-life setting.Methods:Patients with proteinuria >0.5 g/24 h and/or active sediment at baseline enrolled in a multicentre Italian cohort of SLE patients (BeRLiSS study), treated with monthly iv Belimumab 10 mg/kg plus standard of care were considered in this study. Complete renal response (CRR) was defined as proteinuria <0.5 g/24 h, estimated glomerular filtration rate (eGFR)≥90ml/min/1.73m2 and no rescue therapy. Primary efficacy renal response (PERR) was defined as proteinuria ≤0.7 g/24 h, eGFR ≥60ml/min/1.73m2 and no rescue therapy. Prevalence and predictive factors of CRR and PERR at 12 and 24 months after Belimumab initiation were analyzed by multivariate logistic regression analysis.Results:A total of 91 patients were considered in this study, 79 female, mean age 40.51±9.03 years, mean disease duration 12.18±8.15 years, median follow-up time after Belimumab initiation 22 months. Twenty patients had baseline proteinuria ≥0.5 <1 g/day, 17 ≥1 <2 g/day, 13 ≥2 g/day. Belimumab was started at GN onset in 20 (22%) patients and at the time of a renal flare in all other cases. Seventy-five patients underwent a renal biopsy: 1 class I, 4 class II, 14 class III, 47 class IV and 9 class V. Baseline serum creatinine was 82.44±29.26 umol/L; 15 patients showed eGFR<60ml/min/1.73m2 at baseline. Immunosuppresants were taken by 70 (76.9%) patients: 47 micofenolate, 15 azathioprine and 5 ciclosporine. Sixty patients (65.9%) were on antimalarials. During follow-up 34 (37.4%) patients achieved CRR. Among them 5 (14.7%) patients relapsed and 29 (85.3%) patients maintained remission. Mean time to achieved CRR was 9.71±5.91 months.High levels of baseline proteinuria were a negative independent predictor of CRR and PERR at 6 months (OR 0.044 CI95% 0.006-0.320 p=0.002 and OR 0.232 CI95% 0.091-0.596 p=0.002) and 12 months (OR 0.029 CI95% 0.002-0.556 p=0.019 and OR 0.056 CI95% 0.009-0.327 p=0.001). High levels of baseline creatinine were a negative independent predictor of renal response. Renal response at 6 months was a strong predictive factor of renal response at 12 and 24 months.Conclusion:Belimumab is an effective add-on therapy in the treatment of GN in real-life practice setting.Disclosure of Interests:None declared

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