scholarly journals Dermatomyositis Sine Myositis with Membranoproliferative Glomerulonephritis

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Mohammad Bagher Owlia ◽  
Roya Hemayati ◽  
Shokouh Taghipour Zahir ◽  
Mohammad Moeini Nodeh

Dermatomyositis (DM) is an autoimmune disease that is characterized by involvement of proximal musculature and skin. We report a 52-year-old woman with a 6-year history of dermatomyositis sine myositis, who developed lower extremity edema and proteinuria. Pathological examination of renal biopsy showed membranoproliferative glomerulonephritis. She received steroid, cyclophosphamide, and mycophenolate mofetil. Over the 9 to 10 months after the beginning of treatment, the proteinuria was improved.

2018 ◽  
Vol 98 (9) ◽  
pp. 763-766 ◽  
Author(s):  
Jegy M Tennison ◽  
Jack B Fu ◽  
Eduardo Bruera

Abstract Background and Purpose Lower extremity edema, which can be caused by several factors, is often poorly managed with commonly prescribed compression stockings and diuretics. Diuretics are often erroneously given in all forms of edema and may cause problems because their long-term application may induce chronicity of the edema due to disturbance of the renin-angiotensin relationship. Compression therapy, although effective against venous edema, is widely underused. Case Description A 64-year-old man with a history of hypertension, coronary artery disease, psoriasis, and multiple myeloma was admitted to the hospital for neutropenic fever, right lower extremity (RLE) cellulitis, bilateral lower extremity (BLE) weakness, RLE pain, and significant BLE edema. The patient was referred to a lymphedema-certified therapist to apply lower extremity multilayered compression bandaging and document serial limb circumference measurements. Outcomes The patient's weight decreased from 94.5 kg on day 1 of compression bandaging to 86.3 kg on day 7. The circumferences of the affected limbs also decreased. Discussion This case demonstrates the utility of multilayered compression bandaging, typically used in the management of lymphedema, in the control of peripheral edema that is refractory to diuretic therapy.


F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 513
Author(s):  
Syed Shahmeer Raza ◽  
Irfan Ullah ◽  
Saira Kainat Awan ◽  
Muhammad Daniyal Nadeem ◽  
Gulsam Bashir

Primary malignant pericardial mesothelioma (PMPM) is a rare cardiac tumor. The patient usually presents with pericardial constriction, usually misdiagnosed and wrongly managed. We present the case of a 21-year-old woman with a history of pericarditis and cardiac tamponade. The patient was referred from a clinic due to nausea, vomiting and ascites with lower extremity edema, soft and watery diarrhea, and right upper quadrant pain. Surgery (sternotomy and partial pericardiectomy) was proposed after looking at the different relevant investigations; it was not until that the patient was operated on that it was established that this wasn't a mere constriction but a malignancy. The patient shortly died after the operation. Pathology made a diagnosis of PMPM. Along with the classical symptoms those who present with level 1 thoracic adenopathy a decision to operate should be very carefully made, this may lead to a misdiagnosis of PMPM which postoperatively results in patient's death.


2016 ◽  
Vol 19 (1) ◽  
pp. 023 ◽  
Author(s):  
Mehmet Yildirim ◽  
Recep Ustaalioglu ◽  
Murat Erkan ◽  
Bala Basak Oven Ustaalioglu ◽  
Hatice Demirbag ◽  
...  

<strong>Background:</strong> Patients with recurrent pericardial effusion and pericardial tamponade are usually treated in thoracic surgery clinics by VATS (video-assisted thoracoscopic surgery) or open pericardial window operation. The diagnostic importance of pathological evaluation of the pericardial fluid and tissue in the same patients has been reported in few studies. We reviewed pathological examination of the pericardial tissue and fluid specimens and the effect on the clinical treatment in our clinic, and compared the results with the literature. <br /><strong>Methods:</strong> We retrospectively analyzed 174 patients who underwent pericardial window operation due to pericardial tamponade or recurrent pericardial effusion. For all patients both the results of the pericardial fluid and pericardial biopsy specimen were evaluated. Clinicopathological factors were analyzed by using descriptive analysis. <br /><strong>Results:</strong> Median age was 61 (range, 20-94 years). The most common benign diagnosis was chronic inflammation (94 patients) by pericardial biopsy. History of malignancy was present in 28 patients (16.1%) and the most common disease was lung cancer (14 patients). A total of 24 patients (13.8%) could be diagnosed as having malignancy by pericardial fluid or pericardial biopsy examination. The malignancy was recognized for 12 patients who had a history of cancer; 9 of 12 with pericardial biopsy, 7 diagnosed by pericardial fluid. Twelve of 156 patients were recognized as having underlying malignancy by pericardial biopsy (n = 9) or fluid examination (n = 10), without known malignancy previously. <br /><strong>Conclusion:</strong> Recurrent pericardial effusion/pericardial tamponade are entities frequently diagnosed, and surgical interventions may be needed either for diagnosis and/or treatment, but specific etiology can rarely be obtained in spite of pathological examination of either pericardial tissue or fluid. For increasing the probability of a specific diagnosis both the pericardial fluid and the pericardial tissues have to be sent for pathologic examination.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1048.1-1048
Author(s):  
W. Hu

Background:Classical lupus nephritis (LN) is characterized by glomerular immune complex(IC) deposition with glomerular proliferation, basement membrane destruction and cell infiltration. Non-IC mediated renal injury with thrombotic microangiopathy (TMA) was also reported in patients with systemic lupus erythematosus (SLE-renal TMA), but most studies were reported in patients with both LN and renal TMA.Objectives:In this study, clinical features and outcomes of SLE-renal TMA in absence of obvious IC in SLE patients were analyzed.Methods:Patients with glomerular TMA and/or vascular TMA in the absence of obvious subendothelial or epithelial immune deposits were screened out from 2332 biopsied in SLE patients who underwent first renal biopsy from January 2005 to August 2016. Their clinical, histological features and outcomes were retrospectively analyzed.Results:In 2332 renal biopsies obtained from SLE patients, 257 (11.0%) showed renal TMA, of which 237 showed both renal TMA and LN, and 20 biopsies had only renal TMA (SLE-renal TMA). There were 2 males and 18 females with an average age of (25 ± 10) years. The median course of SLE and LN were 3.0(1.0, 6.0) and 0.8(0.5, 1.9) months. All 20 patients deserved acute kidney injury, of which 11 (55%) needed renal replacement therapy (RRT) and 12 (60%) were nephrotic syndrome. Blood system involvement was found in all cases, including 13 cases (65.0%) with TMA triad (microvascular hemolytic anemia, thrombocytopenia and elevated lactate dehydrogenase).Pathological examination showed that 17 cases (85.0%) had both glomerular TMA and vascular TMA. Immunofluorescence and electron microscopy showed that 8 cases (40%) had no IC deposition in glomerulus and 12 cases (60%) had only IC deposition in mesangium. Acute tubulointerstitial lesions in patients requiring RRT were more serious than those no needing for RRT((43.6±24.9) %vs(21.7±20.1) %,P=0.047). The fusion range of foot process was positively correlated with proteinuria (r2= 0.347,P=0.006).All patients received high-dose methylprednisolone pulse therapy. Four patients received plasma exchange and three patients received gamma globulin, respectively. Eleven patients requiring RRT all stop RRT in a median time of 16.0 (9.0, 30.0) days. During a median follow-up of 58.0 (36.0, 92.3) months, complete remission (CR) was obtained in 15 cases, partial remission in 4 cases and no remission in 1 case. Six cases (30%) relapsed. No case died or progressed to end stage renal disease.Conclusion:Renal injury characterized by TMA is not uncommon in SLE renal biopsy cases. The clinical manifestation is special and the renal injury is serious. The renal outcome is good by intensive immunosuppressive therapy. It should be considered as a unique type of renal injury in SLE.References:[1]Moake JL. Thrombotic microangiopathies. N Engl J Med. 2002. 347(8): 589-600.[2]Anders HJ, Weening JJ. Kidney disease in lupus is not always ‘lupus nephritis’. Arthritis Res Ther. 2013. 15(2): 108.[3]Song D, Wu LH, Wang FM, et al. The spectrum of renal thrombotic microangiopathy in lupus nephritis. Arthritis Res Ther. 2013. 15(1): R12.[4]Hu WX, Liu ZZ, Chen HP, Zhang HT, Li LS, Liu ZH. Clinical characteristics and prognosis of diffuse proliferative lupus nephritis with thrombotic microangiopathy. Lupus. 2010. 19(14): 1591-8.[5]Tomov S, Lazarchick J, Self SE, Bruner ET, Budisavljevic MN. Kidney-limited thrombotic microangiopathy in patients with SLE treated with romiplostim. Lupus. 2013. 22(5): 504-9.[6]Li C, Yap D, Chan G, et al. Clinical Outcomes and Clinico-pathological Correlations in Lupus Nephritis with Kidney Biopsy Showing Thrombotic Microangiopathy. J Rheumatol. 2019 .[7]Chen MH, Chen MH, Chen WS, et al. Thrombotic microangiopathy in systemic lupus erythematosus: a cohort study in North Taiwan. Rheumatology (Oxford). 2011. 50(4): 768-75.[8]Park MH, AUID- Oho, Caselman N, Ulmer S, Weitz IC, AUID- Oho. Complement-mediated thrombotic microangiopathy associated with lupus nephritis. Blood Adv. 2018. 2(16): 2090-2094.Disclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 540.3-540
Author(s):  
A. Munir ◽  
C. Sheehy

Background:Corneal melt is a rare inflammatory disease of the peripheral cornea; it may lead to perforation of the globe and visual failure. Corneal melt can be a manifestation of systemic vasculitis in patients with RA and other conditions, such as cancer. Without early and aggressive treatment it may be associated with a poor visual outcome and a high mortality. It has been reported in patients with stable RA.Objectives:A case report in a patient with long standing but well controlled Rheumatoid Arthritis (RA) and metastatic disease.Methods:A 75 year old male with a background of sero positive Rheumatoid Arthritis for more than 10 years presented to the Eye Casualty with a two week history of a painful left red eye. His other medical history was significant for Stage IIB poorly differentiated cancer of the left lower lobe. Left lower lobectomy with a patch of diaphragm resected. Intratumoural lymphovascular invasion noted. He completed Adjuvant Carboplatin/Vinorelbine chemotherapy September, 2017. He had DVT proximal left leg 22ndof September, 2017. Follow up CT in 2018 demonstrated a right renal upper pole lesion for which he was awaiting biopsy with?metastatic lung disease vs primary renal carcinoma. His RA was well controlled on Methotrexate 10mg weekly. He had been treated by the ophthalmology team for left marginal Keratitis for the prior 2 months with steroid eye drops without significant improvement. On presentation to ED, he described sharp eye pain, waking him from the sleep, associated with watery discharge and photophobia. Examination showed corneal melt in left eye involving 25% of inferior portion of the cornea and spastic entropion with injecting eye lashes. He had no active joints and there were no other signs of vasculitis. CRP was 4.1. He had a negative ANA and ANCA; viral swabs were negative. He was admitted under the medical team. Intravenous Methyl Prednisolone was started. The patient felt better after 5 days of Methyl Prednisolone. Left temporary tarsorrhaphy was done by Ophthalmology. Cyclophosphamide was initiated after discussion with Oncologist pending the result of the renal biopsy. Patient was discharged after 5 days of admission in the hospitalResults:The renal biopsy was positive for metastatic Squamous cell carcinoma of lung. Cyclophosphamide was withdrawn and he was started on Carboplatin/Gemcitabine. The corneal melt improved with complete resolution of his visual symptoms.Conclusion:In this case, although the history of RA was felt by the ophthalmology team to be the most likely association with the corneal melt, we would argue the oncological diagnoses were likely the driving force behind the presentation.References:[1]Sule A, Balakrishnan C, Gaitonde S, Mittal G, Pathan E, Gokhale NS, et al. Rheumatoid corneal melt. Rheumatology (Oxford)2002;41:705–6.[2]S. Yano, K. Kondo, M. Yamaguchi et al., “Distribution and function of EGFR in human tissue and the effect of EGFR tyrosine kinase inhibition,” Anticancer Research, vol. 23, no. 5, pp. 3639–3650, 2003.Disclosure of Interests:None declared


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Vanni Valeria Stella ◽  
Villanacci Roberta ◽  
Salmeri Noemi ◽  
Papaleo Enrico ◽  
Delprato Diana ◽  
...  

AbstractPathogenesis of endometriosis is still unclear and a role of both innate and adaptive immune system has been postulated. Some recent findings have revealed an increased risk to have concomitant autoimmune disease in women with endometriosis, but no study so far has investigated whether this association could affect endometriosis severity and stage. We retrospectively reviewed medical patients’ notes of women with a confirmed diagnosis of endometriosis who referred to our endometriosis outpatient clinic between January 2015 and December 2019. Cases (endometriosis and an autoimmune disease) were matched in a 1:3 ratio by age and study period with controls (endometriosis without history of autoimmunity). At univariate logistic analysis, concomitant autoimmunity (OR 2.63, 95% CI 1.64–4.21, p < 0.001) and the number of laparoscopic procedures performed (OR 2.81, 95% CI 1.45–5.43, p = 0.002) emerged as factors significantly associated with the likelihood of stage IV endometriosis. In the multivariate logistic regression model, concomitant autoimmunity remained a significant predictor of stage IV endometriosis (OR 2.54, 95% CI 1.57–4.10, p = 0.004), whereas the association between the number of laparoscopic procedures performed and stage IV endometriosis was found to be of borderline-significance (OR 2.70, 95% 1.37–5.30, p = 0.050). Our findings suggest that endometriosis is more severe in patients who are also affected by autoimmune disturbances after controlling for relevant confounders.


2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Takuji Ota ◽  
Toshiya Kamiyama ◽  
Takuya Kato ◽  
Takayuki Hanamoto ◽  
Kunihiro Hirose ◽  
...  

Abstract Background Hepatic cavernous hemangioma (CH) is the most common hepatic benign tumor. Most cases are solitary, asymptomatic, and found incidentally. In symptomatic cases with rapidly growing tumors and coagulopathy, surgical treatment is considered. In rare cases, diffuse hepatic hemangiomatosis (DHH) is reported as a comorbidity. The etiology of DHH is unknown. Case presentation A 29-year-old female patient had a history of endometriosis treated with oral contraceptives. Hepatic CH was incidentally detected in the segment IVa of the liver according to the Couinaud classification. Follow-up computed tomography (CT) and ultrasound sonography showed the growth of the lesion and formation of multiple new lesions near the first. Enhanced CT and magnetic resonance imaging (MRI) revealed that the new lesions were different from CH. Although oral contraceptives were stopped, all lesions grew in size. Malignancy and possibility of rupture of these tumors were considered due to the clinical course, and we opted for surgical removal of the tumors. Left liver lobectomy and cholecystectomy were performed. Surgical findings were small red spot spreading and a mass in segment IV of the liver. Pathological examination revealed a circumscribed sponge-like tumor with diffuse irregular extension to the adjacent area. Both of the lesions consisted of blood-filled dilated vascular spaces lined by flat endothelium without atypia. The diagnosis was hepatic CH with DHH. The patient was discharged on postoperative day 12 uneventfully. Conclusion We report the successful resection of CH with DHH. The case findings suggest a relationship between oral contraceptive use and enlargement of CH and DHH. Although DHH has been poorly understood, a few previously published cases reported DHH occurrence in patients using oral contraceptives. In such cases, the decision to perform surgical resection should be made after careful examination.


2021 ◽  
Vol 29 (3) ◽  
pp. 409-414
Author(s):  
Kyoung-Bin Min ◽  
Myung-Sung Kang ◽  
Hyun Seo ◽  
Un-Hyo Baek ◽  
Yong-Gon Seo

Author(s):  
Eka Prasetya Budi Mulia ◽  
Kevin Yuwono ◽  
Raden Mohammad Budiarto

Abstract Objectives We aimed to investigate the association between hypertension and asymptomatic lower extremity artery disease (LEAD) in outpatients with known history of coronary artery disease (CAD). Methods Patients with known history of CAD who have been undergone coronary angiography and have significant coronary artery stenosis (more than 60%) were included. LEAD was defined as ankle-brachial index (ABI) < 0.9 in either leg. The risk of LEAD in hypertensive group was analyzed using chi-square test, and correlation between blood pressure (BP) and ABI was analyzed using Pearson correlation test in SPSS v.25. Results One hundred and four patients were included. 82.7% of patients were male. Mean age was 57.05 ± 7.97. The prevalence of hypertension was 35.6%, and the prevalence of LEAD was 16.3%. A higher proportion of LEAD was found in hypertensive (18.9%) compared to non-hypertensive (14.9%), although not statistically significant (OR: 1.33; 95% CI: 0.46 to 3.85; p=0.598). There was an association between ABI and systolic BP (p=0.016), but not with diastolic BP (p=0.102). Conclusions Our study showed that the prevalence of LEAD in hypertension, especially in the CAD population, is relatively high. There was no association between hypertension and LEAD, but a higher prevalence of LEAD was found in hypertensive patients. Nevertheless, LEAD screening is still recommended in hypertensive patients, especially in the CAD population, given the fact that outcomes of health and mortality are worse for those with concomitants of these diseases.


2021 ◽  
Author(s):  
Shima Salehi ◽  
Rozita Hosseini Shamsabadi ◽  
Hassan Otukesh ◽  
Reza Shiari ◽  
Monir Sharafi

Abstract Background: Lupus is an inflammatory and autoimmune disease that involves various tissues and organs of the body. Identification of diagnostic elements to rapid identification of seronegative lupus cases is very important in order to prevent morbidity and progression of disease. This study aimed to compare clinical and laboratory findings of seropositive cases with seronegative lupus patients. Methods: This cross-sectional analytic study was performed on 43 children (17 seronegative and 26 seropositive) with lupus who were admitted to Ali Asghar Hospital during 2007-2017. Seropositive patients had anti-nuclear antibody (ANA) titration >1/80, while seronegative patients had ANA titration <1/80 (at the time of disease diagnosis). Clinical and laboratory findings were compared between two groups.Results: Serositis in patients with ANA- was significantly higher than ANA+ (41.17% vs. 23.07%; p = 0.042). ANA- group had higher autoimmune disease history than ANA+ group (42.85% vs. 15.0%; p = 0.041). The family history of the disease in the ANA- group was greater than ANA+ group (50% vs. 23.52%). The percentage of hypertensive patients in ANA- group was higher than ANA+ group (52.94% vs. 26.92%; p = 0.037). Neurologic symptoms in ANA+ and ANA- groups were 38.46% and 17.64%, respectively (p = 0.043). The frequency of patients with thrombocytopenia in ANA+ group was significantly greater than ANA- group (32% vs. 12.5%; p=0.041). There was no significant difference in other clinical and laboratory findings between two groups. Conclusion: Seronegative lupus patients had higher percentage of musculoskeletal symptoms, autoimmune disease history, familial history of disease, and hypertension, while neurological and thrombocytopenia symptoms were higher in seropositive patients compared to seronegative cases. Therefore, evaluation of these factors can be helpful to diagnosis of seronegative patients.


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