scholarly journals Lupus-related protein-losing enteropathy associated with pseudo–pseudo Meigs’ syndrome and successfully treated with hydroxychloroquine

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Taro Horino ◽  
Masami Ogasawara ◽  
Takeshi Kashio ◽  
Satoshi Inotani ◽  
Masayuki Ishihara ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Course ◽  
Ca 125 ◽  
Related Protein ◽  
Systemic Lupus ◽  
Protein Losing Enteropathy ◽  
First Case ◽  
Potential Case ◽  
Benign Ovarian Tumors

Abstract We herein report the first case of lupus-related protein-losing enteropathy associated with pseudo–pseudo Meigs’ syndrome. Lupus-related protein-losing enteropathy and pseudo–pseudo Meigs’ syndrome are extremely rare complications in patients with systemic lupus erythematosus, Both have a similar clinical course characterized by producing marked ascites, and respond to steroids in typical cases. However, in our case, steroid monotherapy was inadequate and the addition of hydroxychloroquine was effective for their treatment. Furthermore, no reports have previously confirmed elevated CA 125 levels with lupus-related protein-losing enteropathy or increased 99mTc-HSA activity with pseudo–pseudo Meigs’ syndrome. In addition, we are the first to report an evaluation of the histopathology of lupus-related protein-losing enteropathy. Previously reported cases have been described as being caused by either pseudo-Meigs’s syndrome or lupus-related protein-losing enteropathy as the cause of the rare pathology that causes marked pleural effusion and ascites in patients with systemic lupus erythematosus, but It has not been evaluated whether the other is co-occurring. Our case highlights that there is a potential case of overlapping lupus-related protein-losing enteropathy and pseudo-Pseudo-Meigs’s syndrome. Furthermore, it is possible that patients with marked ascites with elevated CA 125 levels were mistakenly diagnosed with Meigs’s syndrome or pseudo-Meigs’s syndrome associated with malignant or benign ovarian tumors and underwent surgery. Clinicians should not forget SLE with pseudo-Pseudo-Meigs’s syndrome as one of the differential diagnoses for marked ascites with elevated CA 125 levels.

scholarly journals Recurrent ascites in systemic lupus erythematosus treated with rituximab - a case report and review of pseudo-pseudo Meigs’ syndrome

2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Sarah Compton ◽  
Sarah Luebker ◽  
DeAnna Baker Frost
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Pleural Effusion ◽  
Large Volume ◽  
Lupus Erythematosus ◽  
Case Reports ◽  
Ca 125 ◽  
Initial Symptom ◽  
Systemic Lupus ◽  
First Case

Systemic lupus erythematosus (SLE) is a chronic autoimmune and inflammatory disease with multisystem consequences. Pseudo-pseudo Meigs’ syndrome (PPMS), or Tjalma syndrome, is a newly recognized manifestation of SLE that is characterized by increased CA-125 level, pleural effusion, and ascites without evidence of tumor. PPMS is relatively rare and likely under-recognized. To our knowledge, there are 11 published case reports about PPMS. In nearly half of the PPMS cases, ascites is the initial symptom of SLE. The pathophysiology of this syndrome is not completely understood but thought to be in part due to chronic inflammation, which is supported by symptoms abating with immunosuppression. We report a case of a 20-year-old woman with known SLE who developed recurrent large volume ascites, subsequently leading to the diagnosis of PPMS, requiring rituximab for additional immunosuppression. To our knowledge, this is the first case of using rituximab as a successful treatment for PPMS.

Longterm Outcomes and Damage Accrual in Patients with Childhood Systemic Lupus Erythematosus with Psychosis and Severe Cognitive Dysfunction

2013 ◽  
Vol 40 (4) ◽  
pp. 513-519 ◽  
Author(s):  
Lily Siok Hoon Lim ◽  
Arlette Lefebvre ◽  
Susanne Benseler ◽  
Earl D. Silverman
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Cognitive Dysfunction ◽  
Lupus Erythematosus ◽  
Clinical Course ◽  
Immunosuppressive Treatment ◽  
Damage Index ◽  
Response To Treatment ◽  
Systemic Lupus ◽  
Damage Accrual

Objective.(1) To describe the clinical course and response to treatment; and (2) to evaluate and compare damage accrual of distinct phenotypic subgroups of patients with clinically important psychiatric illness of pediatric systemic lupus erythematosus (pSLE).Methods.A single-center cohort study of patients with pSLE followed at a pediatric lupus clinic from 1985 to July 2009. Clinical course and response to treatment were studied. Remission was defined by absence of psychiatric/cognitive symptoms while receiving minimal doses of prednisone. Disease activity and damage were measured using SLE Disease Activity Index and SLE Damage Index.Results.Fifty-three children were included: 40 with psychosis and cognitive dysfunction (PSYC group) and 13 with isolated cognitive dysfunction (COG group). All received immunosuppressive treatment. Eighteen of 32 treated with azathioprine required a change to cyclophosphamide for poor response but none on cyclophosphamide required a change. The median times to remission were 72 weeks (PSYC) and 70 weeks (COG). Eight patients (7 PSYC, 1 COG) experienced flare following response/remission. New damage was noted in 50% of children at a median of 11 months: 57% of PSYC group, 31% of COG group. Persistent cognitive dysfunction was seen in 16% of PSYC patients and 15% of COG patients.Conclusion.Most patients responded to immunosuppressive treatment, although median time to remission was > 1 year. Roughly half the patients acquired a new damage item, most of which did not interfere with functional abilities. Fewer than 20% of patients developed neuropsychiatric damage. Both phenotypes of psychiatric pSLE responded equally well to current treatment.

Protein-losing enteropathy associated with systemic lupus erythematosus: response to cyclophosphamide

2004 ◽  
Vol 25 (2) ◽  
pp. 135-138 ◽  
Author(s):  
Gl�ucio R. Werner de Castro ◽  
Simone Appenzeller ◽  
Manoel B. B�rtolo ◽  
L�lian Tereza Lavras Costallat
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Protein Losing Enteropathy

scholarly journals Frequency of SARS-CoV-2 infection and characterization of the clinical course in patients with Systemic Lupus Erythematosus in the city of Manaus-Amazonas

2021 ◽  
Author(s):  
Gabriella Bacellar Marques ◽  
Lucila Cristina Tomé Garcia ◽  
Cecília Tizatto Barroso ◽  
Vitória Miki Pang Takatani ◽  
Samuel Elias Basualto Dias ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Course ◽  
Systemic Lupus ◽  
The City

PROTEIN-LOSING ENTEROPATHY IN SYSTEMIC LUPUS ERYTHEMATOSUS

1988 ◽  
Vol 18 (7) ◽  
pp. 868-871 ◽  
Author(s):  
S. E. J. EDMUNDS ◽  
V. GANJU ◽  
B. R. BEVERIDGE ◽  
M. A. FRENCH ◽  
M. F. QUINLAN
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Protein Losing Enteropathy

Lupus Eritematosus Sistemik pada Pria

2021 ◽  
Vol 3 (2) ◽  
pp. 37-42
Author(s):  
Harry Andrean ◽  
Raveinal Raveinal
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Course ◽  
Multiple Organ ◽  
Systemic Lupus ◽  
Malar Rash ◽  
Mouth Ulcer ◽  
Pulse Dose ◽  
Aggressive Clinical Course ◽  
Complex Autoimmune Disease

Introduction: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by presence of nucleus autoantibody and affected multiple organ. Systemic lupus erythematosus is more common in women than men with ratio 2:1 to 15:1. Men with SLE often have a more aggressive clinical course, lead to a poorer prognosis compared with women with SLE. Case Report: A man, 29 years old came to hospital with main complain joint pain increased since 1 week ago, accompanied with red spot on face, trunk, hands, foot, and back, hair loss, swollen leg, mouth ulcer, and fatique. Malar rash and discoid rash were identified from physical examination. From laboratorium, ANA profile was positive for RNP/Sm, Sm, dsDNA, and histone. Skin biopsy showed a lupus discoid. Conclusion: The patient was treated with pulse-dose methylprednisolone for 3 days and showed a good response clinically.

Successful treatment with belimumab in a patient with refractory systemic lupus erythematosus after initiation of hemodialysis: Considering the synergistic effect of belimumab and immunological burn-out phenomenon in end stage renal disease patients on hemodialysis

2020 ◽  
Author(s):  
Shota Ogura ◽  
Kazunori Karasawa ◽  
Wataru Ono ◽  
Ayaki Ito ◽  
Momoko Seki ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
B Cells ◽  
Renal Disease ◽  
Lupus Erythematosus ◽  
End Stage Renal Disease ◽  
Systemic Lupus ◽  
Transitional B Cells ◽  
First Case ◽  
Stage Renal Disease ◽  
End Stage

Abstract Background: In patients with systemic lupus erythematosus (SLE), disease activity can persist even after initiating dialysis. However, guidelines for the treatment of patients with SLE after dialysis is initiated have not yet been established. Case presentation: We describe the case of a 62-year-old Japanese woman who was diagnosed with SLE at age 12, progressed to end-stage renal disease (ESRD), and initiated hemodialysis for lupus nephritis. However, SLE activity persisted after hemodialysis. Cyclophosphamide and mycophenolate mofetil were administered in addition to prednisolone and immunoadsorption, but this treatment strategy was limited by side effects. The patient was subsequently treated with belimumab, and the activity of SLE decreased rapidly. Conclusions: ESRD patients with SLE show no significant decrease in transitional B cells, and have elevated levels of B-cell activating factor (BAFF). Both transitional B cells and BAFF are important therapeutic targets for belimumab, indicating that patients with ESRD may benefit from belimumab therapy. However, the effects of belimumab may be potentiated in patients with uremia, who may be more susceptible to adverse events such as infections. Patients with SLE who receive belimumab after initiation of hemodialysis therefore require careful follow-up. Here we report the first case of belimumab administration in a patient with SLE after initiation of hemodialysis.

scholarly journals Pseudo-pseudo Meigs’ syndrome: a rare presentation of systemic lupus erythematosus

Reumatismo ◽  
2019 ◽  
Vol 71 (2) ◽  
pp. 108-112 ◽  
Author(s):  
G. Tansir ◽  
P. Kumar ◽  
A. Pius ◽  
S.K. Sunny ◽  
M. Soneja
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pleural Effusion ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Abdominal Distension ◽  
Ca 125 ◽  
Systemic Lupus ◽  
Rare Presentation ◽  
The Past ◽  
History Of

Systemic lupus erythematosus (SLE) is a chronic inflammatory multisystem autoimmune disease. Ascites when associated with pleural effusion and raised CA-125 levels in SLE patient, is known as pseudo-pseudo Meigs’ syndrome (PPMS). This is the case of a 22-year-old lady who presented with complaints of abdominal distension for one month and had a history of spontaneous abortion in the past. Abdominal imaging did not reveal any tumor and after extensive workup a diagnosis of PPMS was made. She was successfully treated with steroids, hydroxychloroquine and cyclophosphamide.

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