scholarly journals Kidney failure after lung transplantation in systemic scleroderma: a case report with literature review

2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Takashi Ishide ◽  
Hiroshi Nishi ◽  
Hiroko Ambe ◽  
Kenjiro Honda ◽  
Motonobu Nakamura ◽  
...  

Abstract Background Systemic scleroderma (SSc) involves multiple organs including the skin, the lung, the kidney, and the esophagus. Nowadays, patient life prognosis has substantially improved due to more appropriate management of lung complications, including lung transplantation. However, the extension of their survival may increase SSc patients with chronic kidney diseases and requiring renal replacement therapy (RRT). Case presentation A 51-year-old female with SSc who underwent unilateral deceased-donor lung transplantation was referred because of progressive renal dysfunction. Despite no episodes of scleroderma renal crisis, her renal function gradually deteriorated for 2 years with her serum creatinine level increasing from 0.5 mg/dL at transplantation to 4.3 mg/dL. Although we reinforced antihypertensive treatment and reduced calcineurin inhibitor dose, she thereafter developed symptomatic uremia. Due to impaired manual dexterity with contracture of the interphalangeal joints, no caregivers at home, and kidney transplantation donor unavailability, maintenance hemodialysis was chosen as RRT modality. Further, due to the narrowing of superficial vessels in the sclerotic forearm skin and post-transplant immunocompromised status, the native left brachiocephalic arteriovenous fistula was created. Post-operative course was uneventful while any sign of cutaneous infection and pulmonary hypertension was closely monitored. Our literature review also indicates several difficulties with initiating and maintaining RRT in patients with SSc although case reports of kidney failure after lung transplanation in SSc were not accumulated. Conclusions With respect to initiating RRT for post-lung transplant patients with SSc, the clinical course of our case exemplifies recent complex trends of renal management. The optimal modality with secured initiation of RRT should be carefully determined based on the severity and risk for the cardiopulmonary, peripheral vascular, cutaneous, and systemic or local infectious complications.

Burns ◽  
2021 ◽  
Author(s):  
Nemanja Baletic ◽  
Robert J. Dabek ◽  
Johanna N. Riesel ◽  
Christopher Hughes ◽  
Martin R. Buta ◽  
...  

2019 ◽  
Vol 40 (Supplement_1) ◽  
pp. S134-S135
Author(s):  
N Baletic ◽  
R J Dabek ◽  
C Hughes ◽  
J N Riesel ◽  
M B Donelan ◽  
...  

2016 ◽  
Vol 6 (2) ◽  
pp. 43-48 ◽  
Author(s):  
Tito Zattera ◽  
Francesco Londrino ◽  
Matteo Trezzi ◽  
Roberto Palumbo ◽  
Antonio Granata ◽  
...  

2021 ◽  
pp. 239719832110139
Author(s):  
Hiral S. Patel ◽  
Vanya Aggarwal ◽  
Rupak Thapa

Introduction: Systemic sclerosis (aka scleroderma) is an autoimmune disease with no known definitive etiology, but genetic, infectious, and non-infectious exposures have been associated with its occurrence. Previous studies have shown that systemic sclerosis can be a paraneoplastic phenomenon and may be associated with increased risk of malignancy, most commonly lung, skin, and breast cancers. The association of renal cell carcinoma with systemic sclerosis is rare. Case Description: Here, we present a case of a 75-year-old male patient with a rapidly progressive scleroderma despite the initial treatment with methotrexate and prednisone 5 mg daily. Shortly after the diagnosis of scleroderma, the patient was found to have a renal mass. The patient underwent a right partial nephrectomy revealing papillary renal cell carcinoma. The surgical margin was negative indicating complete removal of the cancer. The patient, later, developed scleroderma renal crisis and progressed to end-stage renal disease despite treatment with captopril, mycophenolate mofetil, plasmapheresis, and intravenous immunoglobulin. Conclusions: The purpose of this case report and literature review is to highlight that diffuse scleroderma can potentially be paraneoplastic from a renal cell carcinoma and to add more data to the literature given there are only a handful of reported cases. Our patient is unique in the sense that he was discovered to have renal cell carcinoma shortly after being diagnosed with scleroderma suggesting a paraneoplastic etiology yet continued to worsen despite full resection of the renal cell carcinoma. This is in contrast to the other reported cases of renal cell carcinoma associated scleroderma where scleroderma worsened around the time of the diagnosis of renal cell carcinoma and improved after nephrectomy. There also are case reports of the patients with renal cell carcinoma associated scleroderma where the patient had scleroderma for several years before the diagnosis of renal cell carcinoma, which raises the question, if scleroderma could also represent a risk factor for developing renal cell carcinoma.


2002 ◽  
Vol 9 (6) ◽  
pp. 413-416
Author(s):  
Stacey M Pollock-BarZiv ◽  
Marsha M Cohen ◽  
Heather Maclean ◽  
Gregory P Downey

BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare pulmonary disease occurring primarily in women. A literature review of LAM in Canada found sporadic mention of LAM in case reports or within lung transplant studies. The LAM Foundation, a patient support and research funding organization, lists 23 Canadian patients in its database. The present study was designed to assess the scope of LAM across Canada and to identify potential patients for further evaluation. OBJECTIVES: To ascertain Canadian respirologists’ experience with patients with LAM (current and historical), lung transplantation (single or bilateral) and deaths due to LAM, and awareness of the LAM Foundation.METHODS: Four hundred twelve brief surveys were sent anonymously to members of the Canadian Lung Association (inserted in their newsletters) to ascertain the experience of Canadian respirologists with LAM.RESULTS: One hundred twelve surveys were returned (27%). Fifty-one respondents had 'ever' been involved in the care of at least one patient with LAM; eight had cared for three or more patients. At the time of the study, 26 respondents were following a total of 46 patients with LAM; 22 of the 51 respirologists (43%) who had ever cared for a patient with LAM reported the death of a patient. Thirty-three patients had been put on wait lists for transplantation; six died while on the wait list. Nineteen patients underwent lung transplantation - six single-lung recipients and 13 bilateral lung recipients. Of the 51 respirologists who had ever cared for a patient with LAM, only 30 (61%) were aware of the LAM Foundation’s services. Of the 112 respondents, only 47 (43%) were aware of the LAM Foundation.CONCLUSIONS: This study identified a moderate level of awareness of a significant existing patient support and research service (the LAM Foundation). There were many patients with LAM who were unknown to the LAM Foundation and could benefit from its resources. Results suggest that there may be more patients with LAM in Canada than are reported in the existing literature.


Lupus ◽  
2020 ◽  
pp. 096120332096570
Author(s):  
Juliana P Ocanha-Xavier ◽  
Camila O Cola-Senra ◽  
Jose Candido C Xavier-Junior

Reticular erythematous mucinosis (REM) was first described 50 years ago, but only around 100 case reports in English have been published. Its relation with other inflammatory skin disorders is still being debated. We report a case of REM, including the clinical and histopathological findings. Also, a systematic review of 94 English-language reported cases is provided. The described criteria for clinical and histopathological diagnosis are highlighted in order to REM can be confidently diagnosed.


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