Sarcoidosis in Native and Transplanted Kidneys: Incidence, Pathologic Findings, and Clinical Course

Abstract

Renal involvement by sarcoidosis in native and transplanted kidneys classically presents as non caseating granulomatous interstitial nephritis. However, the incidence of sarcoidosis in native and transplant kidney biopsies, its frequency as a cause of end stage renal disease and its recurrence in renal allograft are not well defined, which prompted this study. The electronic medical records and the pathology findings in native and transplant kidney biopsies reviewed at the Johns Hopkins Hospital from 1/1/2000 to 6/30/2011 were searched. A total of 51 patients with a diagnosis of sarcoidosis and renal abnormalities requiring a native kidney biopsy were identified. Granulomatous interstitial nephritis, consistent with renal sarcoidosis was identified in kidney biopsies from 19 of these subjects (37%). This is equivalent to a frequency of 0.18% of this diagnosis in a total of 10,023 biopsies from native kidney reviewed at our institution. Follow-up information was available in 10 patients with biopsy-proven renal sarcoidosis: 6 responded to treatment with prednisone, one progressed to end stage renal disease. Renal sarcoidosis was the primary cause of end stage renal disease in only 2 out of 2,331 transplants performed. Only one biopsy-proven recurrence of sarcoidosis granulomatous interstitial nephritis was identified.ConclusionsRenal involvement by sarcoidosis in the form of granulomatous interstitial nephritis was a rare finding in biopsies from native kidneys reviewed at our center, and was found to be a rare cause of end stage renal disease. However, our observations indicate that recurrence of sarcoid granulomatous inflammation may occur in the transplanted kidney of patients with sarcoidosis as the original kidney disease.

Prognose und Therapie von entzündlich-rheumatischen Erkrankungen

“…Sofortiger ACE-Hemmereinsatz mit konsequent steigender Dosierung auch bei schwer eingeschränkter Nierenfunktion kann Tab. 3 Histopathologisches Klassifikationsschema für ANCA-assoziierte Glomerulonephritiden nach Berden [3] Klasse Einschlusskriterien und Prognose nach 5 Jahren Fokale Läsionen >50% der Glomeruli sind intakt: 5-Jahres Nierenüberleben 93% Halbmonde ≥50% der Glomeruli weisen extrakapilläre Proliferationen (Halbmonde) auf: 5-Jahres-Nierenüberleben 76% Gemischte Läsionen <50% der Glomeruli sind intakt: teilweise bestehen Halbmonde (<50% der Glomeruli), teilweise bestehen vollständig sklerosierte Glomeruli (<50%): 5-Jahres-Nierenüberleben 61% Glomerulosklerose ≥50% der Glomeruli sind global sklerosiert: 5-Jahres-Nierenüberleben 50%, 7-Jahres-Nierenüberleben 25% die renale Krise durchbrechen [47]. Bei schlechter Blutdruckkontrolle ist zusätzli-cher Einsatz von Ca-Antagonisten, α-und β-Blockern und/oder Minoxidil erforderlich [50].…”

“…Bei jeweils 6 (11%) Patienten fanden sich fokal-segmentale oder chronisch fortgeschrittene glomeruläre Sklerose, weiterhin 3 (5%) Immunkomplex-GN, 1 membranöse GN, 1 Amyloidose, 1 Syndrom der dünnen Basalmembranen und 7 (12%) diabetische Nephropathien [3]. Von den 19 Patienten mit granulomatöser Sarkoidose der Nieren wiesen 7 (37%) Patienten eine Hyperkalziämie auf, doch nur in 3 Fällen (16%) fanden sich Mikro verkalkungen im Bioptat.…”

“…Nur in 2-5% der Fälle führt die renale Sarkoidose zum terminalen Nierenversagen. Ein Rezidiv in Transplantatnieren wurde in bis zu 30% beobachtet, sodass die immunsuppressive Erhaltungstherapie nach Nierentransplantation von Sarkoidosepatienten Steroide enthalten sollte [3].…”

Renal allograft granulomatous interstitial nephritis: observations of an uncommon injury pattern in 22 transplant recipients

“…Macrophages in ACR tend to have an infiltrative pattern, and the presence of nodules or discrete aggregates of epithelioid macrophages raises diagnostic consideration of allograft interstitial nephritis from causes other than rejection. Prior reports of allograft GIN have attributed the etiology to bacterial and fungal infections [6, 7] and sarcoidosis [12]. Drug toxicity or hypersensitivity, mycobacterial infections [9, 13–19], other bacterial urinary tract pathogens [9, 20, 21], adenovirus [22–26] and fungal infections [19, 27] have documented associations with native GIN.…”

“…GIN may theoretically arise as a de novo event in the allograft from any of the etiologic agents that cause GIN in native kidneys. Recurrent disease related to sarcoidosis [12, 28–34] or tubulointerstitial nephritis and uveitis (TINU) syndrome [35], are also diagnostic considerations. Rates of graft failure in GIN are highest in Mycobacterium tuberculosis infection [6, 8, 9, 14, 15, 19, 36] with more than 80% graft loss, in contrast to GIN associated with other infections and non-infectious GIN, where graft survival with or without impairment of function, is frequently observed (references in Table 1 and Figure 1 and [37–39]).…”