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Introduction
Renal injury due to sarcoidosis develops in less than a quarter of patients with this systemic disease. In most cases, granulomatous tissue alters the production of vitamin D, which leads to hypercalciuria, nephrocalcinosis, and nephrolithiasis. Granulomatous interstitial nephritis is another well-recognized pathological process associ...
Citations
... E and F Necrotizing vasculitis of a small artery with fibrinoid necrosis (black arrows) (renal histology of biopsy specimen, hematoxylin and eosin staining and elastica-masson staining, original magnification, × 400) sarcoidosis in the proper clinical context [6]. Renal sarcoidosis with vasculitis is unusual, with only four existing reports [7][8][9][10]. The 2012 Chapel Hill Consensus Conference classification listed sarcoid vasculitis as a differential disease associated with systemic illness [11]. ...
Background
Sarcoidosis affects multiple organs and exhibits diverse clinical manifestations. Although tubulointerstitial nephritis is a known feature of renal involvement, necrotizing vasculitis is rare. Furthermore, prostate involvement with urinary retention is unusual in patients with sarcoidosis. Here, we report a case of systemic sarcoidosis with a rare combination of manifestations and different acute kidney injuries.
Case presentation
A 66-year-old man developed sudden urinary retention and fever. He was diagnosed with prostatitis and admitted to our hospital. An indwelling urethral catheter was inserted, and antimicrobial therapy was initiated; however, the prostatitis was refractory. Computed tomography revealed enlarged mediastinal lymph nodes. Analysis of transbronchoscopic lymph node and prostate biopsies showed epithelioid cell granulomas, suggesting systemic sarcoidosis. During the clinical course, the serum creatinine level rapidly increased to 2.36 mg/dL without oliguria. A kidney biopsy revealed tubulointerstitial injury with moderate lymphohistiocytic infiltration and small-vessel vasculitis in the interstitium. Following oral administration of 60 mg/day prednisolone, the patient’s renal function immediately improved, and urinary retention did not recur.
Conclusions
To the best of our knowledge, this is the first reported case of sarcoidosis with two unusual complications. Given its clinical course and pathology, this case is clinically valuable.
... Sarcoidosis is associated with tubulointerstitial nephritis with or without granulomas, nephrolithiasis, nephrocalcinosis, and glomerular diseases and tubular dysfunction. [3][4][5][6][7] Our case is the first report of simultaneous renal AA amyloidosis and crescentic necrotizing glomerulonephritis (CNG) in a patient with sarcoidosis. ...
Sarcoidosis has a rare and independent association with renal AA amyloidosis and crescentic necrotizing glomerulonephritis. However, coexisting entities in sarcoidosis have not been previously described. Herein, we report a 66-year-old Caucasian woman who presented with generalized fatigue, weight loss, and acute kidney injury in the setting of likely sarcoidosis. Renal biopsy revealed AA amyloid fibrils with fibrocellular crescents. The patient's clinical symptoms and laboratory results improved with high-dose glucocorticoids and azathioprine.
... Renal lesions in sarcoidosis cases include lesions associated with abnormal calcium metabolism, glomerular lesions due to the background of systemic inflammatory diseases, and granulomatous interstitial nephritis. As to the glomerular lesions, a variety of different lesions have been described, including membranous nephropathy, IgA nephropathy, minimal-change disease, a proliferative or crescentic glomerulonephritis, and focal segmental glomerulosclerosis [1][2][3]. A case of systemic sarcoidosis with mesangial proliferative glomerulonephritis showing predominant deposition of IgG in the mesangial region is presented. ...
... Renal lesions in sarcoidosis cases include lesions associated with abnormal calcium metabolism, glomerular lesions due to the background of systemic inflammatory diseases, and granulomatous interstitial nephritis [7]. As to the glomerular lesions, a variety of different lesions have been described, including membranous nephropathy, IgA nephropathy, minimal-change disease, a proliferative or crescentic glomerulonephritis, and focal segmental glomerulosclerosis [1][2][3]. A cohort study by Loffler et al. reported that 26% of patients had sarcoidosis with IgA nephropathy, and in all cases, treatment with prednisolone improved renal function and decreased urinary protein [2]. ...
A 37-year-old African–British man was referred to our hospital for detailed examination because of persistent fever, swelling and pain in both ankle joints, and blurred vision for two months. Inguinal lymph node biopsy showed a large number of epithelioid granulomas without necrosis. Granulomatous anterior uveitis, nephropathy, high serum angiotensin-converting enzyme activity, and high serum-soluble interleukin-2 receptor were observed, and the diagnosis of systemic sarcoidosis was made. His serum creatinine was 1.4 mg/dL and hematuria, leukocyturia, and urine protein were also seen. The renal biopsy finding was mesangial proliferative glomerulonephritis, with no findings of granuloma formation or tubular interstitial nephritis. Immunofluorescence staining showed deposition of IgG, C3, and C1q in the mesangial region. IgG3 was dominant in subclass staining. There was no monoclonality on kappa and lambda staining. Electron microscopy showed predominant deposition in the mesangial region with some subepithelial and endothelial deposition. His hematuria and leukocyturia disappeared with steroid therapy, suggesting sarcoidosis-related nephropathy. A case of systemic sarcoidosis with mesangial proliferative glomerulonephritis showing predominant deposition of IgG in the mesangial region is presented. No cases of such histological findings have been reported so far, and it is necessary to analyze further cases to clarify the pathogenic significance of the renal biopsy findings observed in this case.
... In acute allergic drug-induced interstitial nephritis, the major histologic abnormalities are in the interstitium which is expanded by a number of inflammatory cells with the presence of large numbers of eosinophils, as is seen in this patient [7,10,15,18,19]. The true incidence of AZA causing TIN is not very well known. ...
Kidney Transplant is the gold standard care for end-stage kidney disease (ESKD). Immunosuppression has a central role to maintain the graft function and its survival. Despite newer agents, still old agents such as azathioprine is still used by many clinicians at various places worldwide. However, hypersensitivity reactions, such as tubulointerstitial nephritis, can occur after starting these medications. There can be many causes of tubulointerstitial nephritis; drugs, infections and autoimmune diseases e.g.: sarcoidosis, systemic lupus erythematous and Sjogren's syndrome. We report a case of acute interstitial nephritis after introduction of azathioprine in a kidney transplant recipient. A young female with a living related kidney transplant on triple immunosuppression medication consisting of prednisolone, tacrolimus and mycophenolate mofetil, expressed her desire to conceive eight months after her wedding. Accordingly, mycophenolate mofetil was replaced by azathioprine. In subsequent visits it was observed that her graft kidney function was deteriorating progressively. All possible causes which could contribute to graft function deterioration were evaluated and a graft biopsy was performed. The biopsy showed moderate interstitial inflammatory infiltrate rich in eosinophils with no evidence of cellular or antibody mediated rejection. It was successfully treated by stopping azathioprine and starting high dose oral prednisolone. Deterioration of kidney function in kidney transplant patients, who are put on azathioprine, should raise the suspicion of tubulointerstitial nephritis as a differential diagnosis.
... In acute allergic drug-induced interstitial nephritis, the major histologic abnormalities are in the interstitium which is expanded by a number of inflammatory cells with the presence of large numbers of eosinophils, as is seen in this patient [7,10,15,18,19]. The true incidence of AZA causing TIN is not very well known. ...
Abstract
Kidney Transplant is the gold standard care for end-stage kidney disease (ESKD). Immunosuppression has a central role to maintain the graft function and its survival.
Despite newer agents, still old agents such as azathioprine is still used by many clinicians at various places world-wide. However, hypersensitivity reactions, such as tubulointerstitial nephritis, can occur after starting these medications. Tere can be many causes of tubulointerstitial nephritis; drugs, infections and autoimmune
diseases e.g.: sarcoidosis, systemic lupus erythematous and Sjogren’s syndrome.
We report a case of acute interstitial nephritis after introduction of azathioprine in a kidney transplant recipient.
A young female with a living related kidney transplant on triple immunosuppression medication consisting of prednisolone, tacrolimus and mycophenolate mofetil, expressed her desire to conceive eight months after her wedding. Accordingly, mycophenolate mofetil was replaced by azathioprine. In subsequent visits it was observed that her graft kidney function was deteriorating progressively. All possible causes which could contribute to graft function deterioration were evaluated and a graft biopsy was performed. Te biopsy showed moderate interstitial inflammatory infltrate rich in eosinophils with no evidence of cellular or antibody mediated rejection. It was successfully treated by stopping azathioprine and starting high dose oral prednisolone.
Deterioration of kidney function in kidney transplant patients, who are put on azathioprine, should raise the suspicion of tubulointerstitial nephritis as a differential diagnosis.
... Sarcoidosis glomerulonephritis (MGN) [53][54][55][56][57][58][59]. Case reports described singular cases of focal segmental glomerulonephritis [60], membranoproliferative glomerulonephritis [61], minimal change glomerulonephritis [62], and cases of rapidly progressing crescentic glomerulonephritis (RPGN; Fig. 2c) [63,64]. Because of their rare occurrence it can be discussed that these latter forms pose coincidental findings, whereas it is www.co-pulmonarymedicine.com 3 not uncommon that IgA-GN and MGN develop secondary to an underlying disease. ...
... As a consequence of the autonomous expression of 1-alpha-hydroxylase (and therefore the overproduction of 1,25-OH-vitamine D) in pulmonary macrophages, sarcoidosis is often accompanied by hypercalcemia and hypercalciuria, sometimes also isolated hypercalciuria without hypercalcemia. This either leads to the formation of calcium-rich kidney stones [62][63][64][65][66][67][68][69][70][71][72] or to a diffuse calcification of the kidney parenchyma called nephrocalcinosis [15,29,[66][67][68][73][74][75][76]. Renal manifestations based on an impaired calcium homeostasis are associated with a good prognosis because steroid therapy rapidly normalizes serum calcium levels and consecutively improves the renal function. ...
Purpose of review: The purpose of this article is to provide understanding of renal sarcoidosis, the different types of renal sarcoidosis, disease burden of renal involvement, and treatment options.
Recent findings: The frequency of renal involvement seems to be underestimated, but renal sarcoidosis represents a relevant group of organ manifestations and significantly adds to the patient's morbidity. Because histopathological analysis of renal biopsy specimens can reveal various entities, a diagnostic workup is necessary in every patient with sarcoidosis.
Summary: If systematically screened for renal manifestations are likely to occur in up to 25-30% of all sarcoidosis patients. The most common histological form of renal sarcoidosis is the granulomatous interstitial nephritis; however, granulomas can be absent. Furthermore, one can find various forms of secondary glomerulonephritis. In cases with dysregulated calcium homeostasis, nephrocalcinosis and nephrolithiasis are commonly detectable kidney diseases. AA amyloidosis or renal masses because of granuloma formation are considered to be rare manifestations. In addition to glucocorticoids various immunosuppressive treatments such as tumor necrosis factor alpha inhibitors have proven to be effective based on case series.
... Die wichtigsten Formen einer RS sind: 4 interstitielle Nephritis j mit Nachweis von Granulomen j ohne Nachweis von Granulomen 4 Hyperkalzämie-bedingte Erkrankungen j Nephrokalzinose (NC) j Nephrolithiasis (NL) 4 sekundäre Glomerulonephritiden j IgA-Glomerulonephritis (IgAN) j membranöse Glomerulonephritis j sonstige Glomerulonephritiden Extrem selten und nur in Einzelfällen beschrieben sind glomeruläre Manifestationsformen wie die Minimal-change-Glomerulonephritis (MCGN), die fokal-segmentale Glomerulosklerose (FSGS) sowie die rapid-progressive Glomerulonephritis mit Halbmondbildung (RPGN) [27,33]. . Abb. 4 informiert über die Häufigkeiten der verschiedenen Formen. ...
Renale Beteiligungen der Sarkoidose sind wesentlich häufiger als in alten epidemiologischen Studien angenommen. Sie verursachen jedoch in den meisten Fällen wenige Symptome und fallen erst bei gezielter Diagnostik auf. Im Rahmen eines kompletten Organstagings bei Sarkoidose sollte eine gezielte Diagnostik in Richtung einer möglichen Nierenbeteiligung nicht fehlen. Bei Hinweisen für eine Nierenbeteiligung in der Labordiagnostik wie Proteinurie, auffälligem Urinsediment oder einer eingeschränkter Nierenfunktion sollte zur Klärung der Art, Schwere und der Prognose der renalen Sarkoidose großzügig die Indikation zur Nierenbiopsie gestellt werden. Die Therapie stützt sich in erster Linie auf die Gabe von Kortikosteroiden. Zur Einsparung von Steroiden können immunsuppressive Basistherapeutika und Infliximab eingesetzt werden. Die Evidenz für diese Therapien beruht jedoch weitgehend auf dem Level von Fallberichten und Expertenmeinungen. Nach Absetzen der Immunsuppression besteht ein hohes Risiko für Rezidive.
... Successful achievement of remission in case of crescentic necrotizing glomerulonephritis related to sarcoidosis with use of azathioprine along with prednisone therapy tapering was reported [53]. The recommended dose of azathioprine for renal sarcoidosis is 2 mg/kg/day [41]. ...
Sarcoidosis remains to be an enigma in contemporary medicine. Spectrum of renal manifestations of sarcoidosis is extensive and fascinating. Electrolyte abnormalities, including hypercalcemia and hypercalciuria can present in acute and chronic fashion. These can result in the development of acute neurological symptoms of confusion and neuromuscular disturbances and present in the form of chronic renal tubular dysfunctions. Parenchymal involvement of the kidney in sarcoidosis varies from chronic granulomatous interstitial nephritis to extensive spectrum of glomerular diseases, including membranous glomerulonephritis, crescentic glomerulonephritis, IgA nephropathy, focal segmental glomerular sclerosis, and minimal change disease. The mass-like appearance of renal sarcoidosis may mimic malignant tumors and lymphomas. Diagnosis of renal sarcoidosis requires exclusion of other conditions and thorough search for extrarenal manifestations to support it. Treatment of sarcoidosis includes controlling of electrolyte disturbances and immunosuppression for end-organ diseases. The unique immunological interface of the kidneys frequently dictates more aggressive and extensive immunosuppression to prevent relapse of parenchymal and glomerular diseases. In this chapter, we review the epidemiology of renal sarcoidosis and diverse presentations of sarcoidosis encountered in medicine and nephrology practice. Thereafter, we will discuss the approach to the differential diagnoses of hypercalcemia and hypercalciuria. Later, we will describe the glomerular pathology encountered in patients with sarcoidosis. Finally, we will review the current literature on the treatment of hypercalcemia and use of immunosuppressive agents for interstitial and glomerular kidney diseases related to sarcoidosis.
... glomerulonephritis (MGN) [53][54][55][56][57][58][59]. Case reports described singular cases of focal segmental glomeru- lonephritis [60], membranoproliferative glomerulo- nephritis [61], minimal change glomerulonephritis [62], and cases of rapidly progressing crescentic glomerulonephritis (RPGN; Fig. 2c) [63,64]. Because of their rare occurrence it can be discussed that these latter forms pose coincidental findings, whereas it is www.co-pulmonarymedicine.comnot ...
... As a consequence of the autonomous expression of 1-alpha-hydroxylase (and therefore the overproduc- tion of 1,25-OH-vitamine D) in pulmonary macro- phages, sarcoidosis is often accompanied by hypercalcemia and hypercalciuria, sometimes also isolated hypercalciuria without hypercalcemia. This either leads to the formation of calcium-rich kidney stones [62][63][64][65][66][67][68][69][70][71][72] or to a diffuse calcification of the kidney parenchyma called nephrocalcinosis [15,29,[66][67][68][73][74][75][76]. Renal manifestations based on an impaired calcium homeostasis are associated with a good prognosis because steroid therapy rap- idly normalizes serum calcium levels and consecu- tively improves the renal function. ...
Hypokalemia and hyperkalemia are among the most common disturbances of the electrolyte status.
Since the regulation of the potassium homeostasis depends on normal renal function, patients with acute or chronic renal disease tend to develop such disturbances. The clinical consequences of hypokalemia or hyperkalemia are often severe and unforeseen, which is illustrated by respective case reports in this paper.
Due to the often severe clinical consequences of hypokalemia or hyperkalemia, these electrolyte disturbances deserve special diagnostic and therapeutic attention.
