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

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.

Rheumatology ◽  
2017 ◽  
Vol 56 (6) ◽  
pp. 957-964 ◽  
Author(s):  
Constance Jensina Ulff-Møller ◽  
Jacob Simonsen ◽  
Kirsten Ohm Kyvik ◽  
Søren Jacobsen ◽  
Morten Frisch

2021 ◽  
Vol 3 (1) ◽  
pp. 73-82
Author(s):  
Limdawati Kwee

The systemic lupus erythematosus (SLE) is an autoimmune disease characterized by theformation of autoantibodies that attack various organ systems. Patients with SLE who presentwith pleural effusion are so rare that they are often not diagnosed early. Whereas with earlydiagnosis and proper management, the rate of recurrence and complications can decrease, thequality of life for people with SLE will also be better. This research is descriptive qualitativeresearch with a case study approach. This case report discusses a young woman who presentedwith respiratory failure resulting from a massive pleural effusion. The patient is known to have ahistory of chronic hematological disorders in the form of thrombocytopenia. During treatment,the patient developed psychiatric disorders in the form of delirium and hallucinations. The patientwas then diagnosed as SLE based on the 2018 EULAR / ACR criteria with the degree of disease activity classified as severe. The patient responded well to steroid induction therapy,azathioprine, and hydroxychloroquine. It was concluded that the diagnosis of SLE should beconsidered in cases of pleural effusion with a history of chronic thrombocytopenia and psychiatricdisorders. Early and appropriate management of severe SLE will provide a good prognosis. Keywords: systemic lupus erythematosus; respiratory failure; pleural effusion; neuropsychiatricSLE


2021 ◽  
Vol 3 (1) ◽  
Author(s):  
Sarah Compton ◽  
Sarah Luebker ◽  
DeAnna Baker Frost

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.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Zahedin Kheyri ◽  
Amirreza Laripour ◽  
Moein Ala

Abstract Background Systemic lupus erythematosus is an inflammatory disease affecting several organs. Serositis is one of the systemic lupus erythematosus presentations, but peritonitis is a relatively rare presentation. Particularly, it is extremely rare to observe peritonitis as the first presentation of systemic lupus erythematosus. Case presentation Here, we present a case of peritonitis without other symptoms of systemic lupus erythematosus, in a patient who was finally diagnosed with systemic lupus erythematosus. Our patient was a 27-year-old Persian/Caucasian male with fatigue, weakness, weight loss, abdominal distension, massive ascites, and normocytic hemolytic anemia. He did not mention any prior medical conditions and did not use any drugs. There were no signs of thyroid dysfunction, cardiac dysfunction, cancers, infectious diseases, hepatitis, kidney diseases, or other diseases. Low-gradient, high-protein ascites fluid, and positive antinuclear antibody and anti-double stranded DNA were in favor of systemic lupus erythematosus. Corticosteroid pulse therapy led to resolution of ascites, and the patient was discharged with prednisolone and hydroxychloroquine. Conclusion Peritonitis is a rare presentation of systemic lupus erythematosus, particularly as the first presentation and in the absence of other signs and symptoms; however, systemic lupus erythematosus should be considered as one the differential diagnoses for peritonitis when other etiologies have been ruled out.


Author(s):  
Sunita Dubey ◽  
Poonam Goel ◽  
Nidhi Pandey ◽  
Pavithra H. N.

Serosal fibroid of uterus are usually asymptomatic but rarely; it may present with atypical symptoms to simulate malignancy and needs extensive evaluation. Authors are reporting a case of 26 years old P1L1 female with history of asymptomatic multiple intramural fibroids since 6 years came with complained of progressively increasing abdominal distension and mild pain abdomen from 2-3 months. On evaluation, she had ascites, pleural effusion and raised Ca-125. MR imaging of pelvis revealed moderate ascites and pedunculated serosal fibroid in addition to intramural fibroids with normal bilateral ovaries. She was evaluated to rule out uterine sarcoma and tuberculosis but diagnosis of them could not be established. Finally, conclusion of Pseudo-Meigs syndrome was made. Myomectomy of single pedunculated fibroid relieved her symptoms. Though, subserosal fibroids are benign in pathology, timely surgery is must to avoid morbidity and mortality owing to massive ascites and pleural effusion.


2016 ◽  
Vol 2016 ◽  
pp. 1-2
Author(s):  
Ali Naderi Mahabadi ◽  
Bassam Alhaddad ◽  
Stanley Ballou

Cardiac involvement is fairly common in patients with systemic lupus erythematosus (SLE). It may involve all layers of the heart and coronary arteries as well as the heart valves. We report an extremely rare presentation of valvulitis and valvular dysfunction associated with systemic lupus erythematosus. This is the first case of lupus valvulitis which required three mechanical prosthetic valve replacements with disease recurrence leading to a fatal outcome. This is, in our point of view, the consequence of aggressive natural history of the disease and perhaps late diagnosis and treatment of underlying SLE which was unsuccessful.


Author(s):  
Siavash Abedi ◽  
Ali Sharifpour ◽  
Masoud Aliyali ◽  
Fatemeh Niksolat ◽  
Seyyed Abbas Hashemi ◽  
...  

Systemic Lupus Erythematosus (SLE) known as “lupus” is an autoimmune disease that mostly affects women. In this disorder, the body’s immune system attacks different tissues.Manifestations and symptoms vary among individuals from mild to severe. One of the most common manifestations of lupus is pleuritis with pleural effusion; however, its manifestationas the initial symptom of the disease is remarkably rare. Herein, we present a 17-year-old female with SLE, which was diagnosed with early pleural effusion regarding rheumatologiccounseling subjected to treatment


2020 ◽  
Vol 13 (1) ◽  
pp. e229382
Author(s):  
Tiago Gama Ramires ◽  
Luísa Vieira ◽  
Nuno Riso ◽  
Maria Francisca Moraes-Fontes

A 23-year-old woman with fever, oral ulcers, arthalgias and weight loss of 2-week duration suddenly developed blurred vision, with reduced visual acuity, cotton wool exudates and retinal vascular tortuosity. Laboratory testing revealed anaemia, lymphopaenia, positive antinuclear antibody and high anti-dsDNA antibody titre with low complement components. There was no evidence of infection, clinching the diagnosis of lupus retinopathy. Steroid therapy alone was highly effective and was also accompanied by a normalisation of haemoglobin and lymphocyte counts, after which azathioprine was added. Hydroxychloroquine was introduced after resolution of retinal changes. Immunosuppressive therapy was progressively tapered over the course of 12 months and then discontinued, and the patient remains in remission 48 months after the initial presentation. Our patient exemplifies a very rare manifestation of systemic lupus erythematosus. We emphasise the importance of its early detection and complexity of treatment in order to reduce visual morbidity.


Lupus ◽  
2021 ◽  
pp. 096120332110103
Author(s):  
Alfonso Ragnar Torres Jimenez ◽  
Nayma Ruiz Vela ◽  
Adriana Ivonne Cespedes Cruz ◽  
Alejandra Velazquez Cruz ◽  
Alma Karina Bernardino Gonzalez

Shrinking Lung Syndrome (SLS) is a rare and little known complication associated with Systemic Lupus Erythematosus (SLE), characterized by progressive and unexplainable dyspnea, pleuritic pain, small pulmonary volumes and elevation of the diaphragm on chest X-rays as well as restrictive pattern on pulmonary function tests. Objective To describe clinical, radiological and treatment characteristics in pediatric patients with SLS. Material and methods This is a descriptive and retrospective study in patients under 16 years old with the diagnosis of SLE complicated by SLS at the General Hospital. National Medical Center La Raza. Clinical, radiological and treatment variables were analyzed. Results are shown in frequencies and percentages. Results Data from 11 patients, 9 females and 2 males were collected. Mean age at diagnosis of SLS was 12.2 years. Age at diagnosis of SLE was 11.1 years. SLEDAI 17.3. Renal desease 72%, hematological 91%, lymphopenia 63%, mucocutaneous 72%, neurological 9%, arthritis 54%, serositis 91%, fever 81%, secondary antiphospholipid syndrome, low C3 72%, low C4 81%, positive ANA 91%, positive anti-DNA 91%. Regarding clinical manifestations of SLE: cough 81%, dyspnea 91%, hipoxemia 81%, pleuritic pain 71%, average oxygen saturation 83%. Chest X-rays findings: right hemidiaphragm affection 18%, left 63%, bilateral 18%. Elevated hemidiaphragm 91%, atelectasis 18%, pleural effusion 91%, over one third of the cardiac silhouette under the diphragm 36%, bulging diaphragm 45%, 5th. anterior rib that crosses over the diaphragm 91%. M-mode ultrasound: diaphragmatic hypomotility 100%, pleural effusion 63%. Pulmonary function tests: restrictive pattern in 45% of the cases. Treatment was with supplementary oxygen 100%, intubation 18%, antibiotics 100%, steroids 100%, intravenous immunoglobulin 54%, plasmapheresis 18%, cyclophosphamide 54% and rituximab 18%. The clinical course was favorable in 81%. Conclusions SLS should be suspected in patients with SLE and active disease who present hipoxemia, pleuritic pain, cough, dyspnea, pleural effusion and signs of restriction on chest X-rays. Therefore, a diaphragmatic M-mode ultrasound should be performed in order to establish the diagnosis.


Lupus ◽  
2021 ◽  
pp. 096120332098345
Author(s):  
Alessandra Ida Celia ◽  
Roberta Priori ◽  
Bruna Cerbelli ◽  
Francesca Diomedi-Camassei ◽  
Vincenzo Leuzzi ◽  
...  

Proteinuria is one of the most typical manifestations of kidney involvement in Systemic Lupus Erythematosus (SLE). We report the case of a 23-year-old woman with a 6-year-long history of SLE presenting with proteinuria after a three-year remission on hydroxychloroquine. Kidney histological examination showed alterations inconsistent with lupus nephritis and suggestive of hydroxychloroquine toxicity or Fabry disease. The latter was confirmed by genetic assay.


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