Renal Involvement In Sarcoidosis Research Articles

MULTISYSTEMIC SARCOIDOSIS: AN UNUSUAL PRESENTATION

TYPE: Case Report TOPIC: Pulmonary Manifestations of Systemic Disease INTRODUCTION: Sarcoidosis is a chronic multisystemic disease characterized by noncaseating granulomas which affects the pulmonary system and intrathoracic lymph nodes most frequently. Pleural and renal involvement in sarcoidosis is rare. CASE PRESENTATION: A 34 year old African American female with presented with progressively worsening shortness of breath. Of note, this was her fourth hospitalization in six months. Her first presentation was for flank pain. Workup revealed a renal infiltrative process and a left sided pleural effusion. Kidney biopsy was significant for noncaseating granulomas. A right lower lobe pulmonary embolus (PE) was diagnosed on her second admission and recurrent left sided pleural effusions were noted on the third and fourth admissions. Hepatic infiltrative hypodensities in the liver were visualized. Angiotensin converting enzyme level was 137 U/L. On her final presentation, bronchoscopy demonstrated a stereotypical cobblestone appearance and transbronchial lymph node biopsy demonstrated non-necrotizing granulomatous disease. DISCUSSION: Most cases of sarcoidosis tend to affect the lungs, however, both pleural effusion or granulomatous interstitial nephritis (GIN) as a presenting symptom of the disease is uncommon. Pleural involvement is sarcoidosis occurs in 0.7-10%. Clinically relevant GIN is identified in 0.5-0.9% of native kidney biopsies making it a rare histological diagnosis. With workup for other pathology being negative, sarcoidosis was assessed as the cause for the unique clinical course of this patient with biopsy proven pleural and renal disease, and possible hematologic, ocular, and hepatic involvement. CONCLUSIONS: Clinicians must maintain a high level of suspicion for sarcoidosis in patients with multisystemic disease, due to the potential for atypical presentations. DISCLOSURE: Nothing to declare. KEYWORD: Sarcoidosis

Renal involvement in sarcoidosis

Sarcoidosis is a systemic granulomatosis that affects the lungs and mediastinum. Renal involvement is rare in this disease. It's most commonly granulomatous interstitial nephritis. The most common form of presentation is naked renal failure. This is a retrospective study that collected 12 cases of sarcoidosis complicated by acute renal failure. The study consisted of identifying the epidemiological, clinical, biological and histologic data that confirmed the diagnosis from January 1994 to January 2016. A search for extra-renal placement of sarcoidosis concerned all cases. Then, a study of the therapeutic and evolutionary profile was carried out in all cases. Twelve patients (8 males and 4 females) with a mean age of 43 years. The intradermal tuberculin reaction sit negative in 6 cases. Biological analysis showed acute renal failure with a median creatinine of 54.32 mg/L, biological inflammatory syndrome in all cases, normal blood calcium in 9 cases, hypercalcaemia in 6 cases, hypercalciuria in 4 cases, hypergammaglobulinemia of polyclonal appearance in 3 cases and elevated proteinuria in 2 cases. Renal ultrasound was normal in all patients. The renal biopsy performed in all cases showed tubulointerstitial nephropathy. The extra-renal signs found were pulmonary interstitial syndrome in 4 cases, dry syndrome in 4 cases, mediastinal lymphadenopathy in 4 cases, one patient presented a unilateral uveitis. Two patients underwent extra-renal treatment. All patients were on corticosteroids with a clear improvement in creatinine and normalisation of renal function in all patients. The average follow-up time was 18 months. Renal involvement during sarcoidosis is rare (10%) but may progress to renal failure (3% of cases). The renal biopsy is of great interest to maintain the diagnosis of sarcoidosis, especially in the absence of other extra-renal signs. Renal involvement during sarcoidosis is rarely revealing and may delay diagnosis, leading to chronic and even end-stage renal disease.

POS-200 SARCOID TUBULO-INTERSTITIAL NEPHRITIS: A REVIEW OF SIX CASES AT ONE CENTER

Sarcoidosis is an idiopathic multisystem granulomatous disease of uncertain cause. It mostly affects lungs and lymph nodes. Renal involvement in sarcoidosis is rare. It is usually due to abnormal calcium balance or parenchymal involvement, which may ultimately cause renal dysfunction. The aim of our study was to analyze the epidemiologic, clinical, paraclinical, therapeutic features, and the outcome of sarcoidosis in patients with renal manifestations.

Renal involvement in sarcoidosis: histological patterns and prognosis, an Italian survey.

Background:Granulomatous interstitial nephritis in sarcoidosis (sGIN) is generally clinically silent, but in <1% causes acute kidney injury (AKI).Methods:This Italian multicentric retrospective study included 39 sarcoidosis-patients with renal involvement at renal biopsy: 31 sGIN-AKI, 5 with other patterns (No-sGIN-AKI), 3 with nephrotic proteinuria. We investigate the predictive value of clinical features, laboratory, radiological parameters and histological patterns regarding steroid response. Primary endpoint: incident chronic kidney disease (CKD) beyond the 1°follow-up (FU) year; secondary endpoint: response at 1°line steroid therapy; combined endpoint: the association of initial steroid response and outcome at the end of FU.Results:Complete recovery in all 5 No-sGIN-AKI-patients, only in 45% (13/29) sGIN-AKI-patients (p=0.046) (one lost in follow-up, for another not available renal function after steroids). Nobody had not response. Primary endpoint of 22 sGIN-AKI subjects: 65% (13/20) starting with normal renal function developed CKD (2/22 had basal CKD; median FU 77 months, 15–300). Combined endpoint: 29% (6/21) had complete recovery and final normal renal function (one with renal relapse), 48% (10/21) had partial recovery and final CKD (3 with renal relapse, of whom one with basal CKD) (p=0.024). Acute onset and hypercalcaemia were associated to milder AKI and better recovery than subacute onset and patients without hypercalcaemia, women had better endpoints than men. Giant cells, severe interstitial infiltrate and interstitial fibrosis seemed negative predictors in terms of endpoints.Conclusions:sGIN-AKI-patients with no complete recovery at 1°line steroid should be treated with other immunosuppressive to avoid CKD, in particular if males with subacute onset and III stage-not hypercalcaemic AKI.

Renal involvement in sarcoidosis: Histologic findings and clinical course

Background: Renal failure in sarcoidosis is rare and data on its long-term outcome are scarce. Aim: To investigate the pattern of renal involvement in sarcoidosis, its clinical course and response to treatment in the long-term. Methods: A single-center retrospective study with review of renal biopsies and medical charts was performed. Results: Between January 2005 and December 2016, seven patients with sarcoidosis underwent a kidney biopsy. This is equivalent to a frequency of 1.6% in a total of 434 biopsies from native kidney performed in our institution. All patients presented with renal failure. Five patients had granulomatous interstitial nephritis (GIN) and one patient each interstitial nephritis without granuloma and nephrocalcinosis. Three patients had concomitant glomerular disease: IgA nephropathy (n = 2), membranous and focal proliferative glomerulonephritis (n = 1). Most patients (n = 5) presented with hypercalcemia. All patients initially received oral prednisolone 1 mg/kg/day (n = 3) or 0.5 mg/kg/day (n = 4), respectively, with subsequent tapering or suspension. One patient was started on azathioprine after 18 months to spare steroids. After a mean follow-up of 59 months mean estimated glomerular filtration rate (eGFR) had improved from 19 ± 7 at presentation to 49 ± 16 mL/min. No patient required dialysis. All patients started on prednisolone 1 mg/kg/day developed transient diabetes mellitus while patients on 0.5 mg/kg/day did not. Renal function improvement did not differ between both treatment groups. Conclusion: GIN was the most common diagnosis in sarcoidosis patients with renal failure. Initial hypercalcemia was observed in the majority. Early steroid treatment lead to sustained renal function improvement.

Sarcoidosis : Renal manifestations

Renal involvement in sarcoidosis is much more common than generally assumed from old epidemiological studies and is often only detected when actively searched for. Many patients with renal sarcoidosis present with no or only few symptoms. The diagnostic work-up of sarcoidosis should always include apossible renal involvement. In cases of impaired renal function, proteinuria or a pathological urine sediment, arenal biopsy specimen should be obtained to assess the type, severity and prognosis of the kidney disease. Treatment is primarily based on the use of corticosteroids. Steroid-sparing agents, such as disease-modifying antirheumatic drugs and infliximab can be applied; however, the evidence for efficacy of these therapies is mostly based on case series and expert opinions. Discontinuation of immunosuppression therapy bears ahigh risk of relapse.

Sarcoïdose systémique et glomérulonéphrite extra-membraneuse

Renal involvement in sarcoidosis is rare and more often related to calcium metabolism disorders or granulomatous interstitial nephritis. Glomerulonephritis is exceptional. There may be a long latency period between the development of active sarcoidosis and glomerular involvement and inversely. We report a case membranous glomerulonephritis revealing systemic sarcoidosis.

Interstitial and glomerular renal involvement in sarcoidosis

Interstitial and glomerular renal involvement in sarcoidosis

L’insuffisance rénale au cours de la sarcoïdose

Renal sarcoidosis is rare and may lead to renal failure in less than 3% of patients. It may occur as a consequence of calcium metabolism disorders or granulomatous interstitial nephritis. In this retrospective study, we present five patients with renal failure secondary to sarcoidosis diagnosed in our centre on one period of nine years. Patients were three males and two females with a mean age at the time of the diagnosis of 31,6 years. Pulmonary involvement was present in three cases. Renal biopsy revealed granulomatous interstitial nephritis lesions in all patients. Extra-membranous glomerulonephritis was present in one case. In another case, moderate interstitial fibrosis was observed. Corticosteroid therapy using prednisolone 1mg/kg per day was used in all patients. Three patients had methylprednisolone pulse before oral corticosteroid therapy. One patient required several sessions of haemodialysis. All patients were followed up for a mean period of 52,6 months (ranged from 13 to 84 months). All patients improved their renal function with normalization of creatininemia in two of them. Renal involvement in sarcoidosis is probably underestimated. Corticosteroids therapy is efficient and must be introduced early to prevent progression to chronic renal failure.

Sarcoidosis and the kidney: not only the granulomatous interstitial nephritis

Renal involvement in sarcoidosis displays a wide range of manifestations, and kidney dysfunction may involve all three mechanisms of renal failure. We report a new case of systemic sarcoidosis presenting as a severe renal failure due to hypercalcemia, sarcoidosis-related bilateral nephrolithiasis and granulomatous interstitial nephritis. A prostate adenocarcinoma was also diagnosed, but has to be regarded as an unrelated disease.

Non-granulomatous Interstitial Nephritis in a Chinese Man with Sarcoidosis

Clinical renal involvement in sarcoidosis is rare and has not been previously reported in Chinese patients. We report a case of non-granulomatous interstitial nephritis that presented with acute renal failure in a Chinese man with underlying sarcoidosis. Use of prednisolone led to dramatic renal improvement and partial resolution of his asymptomatic lung parenchymal lesions. Unfortunately, the patient subsequently died of cryptococcal meningitis and episodes of nosocomial pneumonia. One should closely monitor a patient with a presumptive diagnosis of sarcoidosis after embarking on treatment since infections like tuberculosis may mimic or coexist with the disease. This is particularly important in areas where sarcoidosis is exceedingly rare.

Sarcoïdose et insuffisance rénale: à propos de deux cas

Renal involvement in sarcoidosis is rare and more often related to calcium metabolism disorders or granulomatous interstitial nephritis, glomerulonephritis is exceptional. The two cases of renal failure reported in this article illustrate the gravity of this complication, whose treatment remains difficult.

Les manifestations rénales de la sarcoïdose. à propos de neuf observations

Clinical renal involvement in sarcoidosis (report of nine cases). Purpose. — Clinical renal outbreaks occurring in the course of sarcoidosis are polymorphous. Methods. — Nine patients presenting with sarcoidosis were followed up for 18 years. Results. — Five patients presented with chronic interstitial nephritis. Renal failure accompanying granuloma was also present in three of them. Corticotherapy allowed rapid improvement in renal function in three patients. In two other cases, late treatment prevented recovery and led to end-stage renal failure in one case. In another case, persistent hypercalciuria was responsible for bilateral nephrolithiasis further treated via extracorporeal lithotrity. One case of mesangial glomerulonephritis and two morbid associations (retroperitoneal fibrosis and Henoch-Schönlein purpura) were observed. Conclusion. — Interstitial nephritis is still a severe clinical renal outbreak. Corticotherapy must be prescribed early to avoid renal failure. Calcium metabolism disorders are frequent and often combined with interstitial nephritis. Hypercalcemia can often and rapidly be improved via corticotherapy, while monitoring of hypercalciuria proves to be more difficult. Membranous glomerulonephritis is still the most frequently reported glomerular lesion.

RENAL DISEASE AND SARCOIDOSIS

Renal involvement in sarcoidosis has recently been emphasized with increasing reports of associated glomerulonephritis. We report in detail a further case of mesangiocapillary glomerulonephritis in association with sarcoidosis. In addition, a review of 75 cases of sarcoidosis at Royal Melbourne Hospital over a 10-year period revealed one case of membranous glomerulonephritis and one case of granulomatous interstitial nephritis. Seven patients in the series had nephrocalcinosis, and eight further patients had abnormalities of urinary sediment of clinical symptoms of renal involvement. These findings suggest that, though renal failure is rare, renal involvement in sarcoidosis is more frequent than has previously been reported.

Renal involvement in sarcoidosis.

Summary Among 152 sarcoidosis patients, 11% had hypercalcaemia, 62% had hypercalciuria and 13·8% had at least one renal stone. Impairment of renal function was mostly conspicuous in patients with hypercalcaemia. Twenty-five successful percutaneous renal biopsies were performed. Epithelioid granulomas were seen in ten cases, with inflammatory reaction more prominent than in other organs involved by sarcoidosis, due to simultaneous pyelonephritis. Thickening and hyalinization of capsular membrane and glomeruli as well as arterial walls were frequently encountered. Amyloid deposits could be seen in one case with chronic lung infection. Histopathological changes specific of sarcoidosis are related to an abnormal amount of serum gammaglobulins and calciuria exceeding 200 mg in 24 hr, which seem to be the best tests of involvement by the disease. Tubular reabsorption of calcium was low in two patients with hypercalciuria, which was corrected after corticosteroid treatment. High intestinal calcium absorption was lowered after hydrochlorothiazide had decreased urine calcium.