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Renal Sarcoidosis

Renal Interstitial Sarcoidosis


Presentation

  • Most common mode of presentation was acute renal failure (11) during spring/summer (14). Evidence for systemic disease was present in all patients. Mean plasma creatinine on presentation was 0.52 mmol/L and calcium 3.01 mmol/L.[ncbi.nlm.nih.gov]
  • A complete response to therapy at 1 year was strongly correlated with hypercalcemia at presentation (p 0.003).[ncbi.nlm.nih.gov]
  • Abstract Sarcoidosis commonly presents in young adults with bilateral hilar lymphadenopathy, lung parenchymal disease and/or skin lesions.[ncbi.nlm.nih.gov]
  • Antecedent history of pneumonia was present in one, and ASO titer was elevated in the other. The proteinuria and azotemia improved in both with steroid therapy.[ncbi.nlm.nih.gov]
  • Secondly, we present follow-up data over a period of 2 years or more.[ncbi.nlm.nih.gov]
Apyrexia
  • Clinical examination found a preserved general state, apyrexia and normal blood pressure in all patients. No cases of peripheral lymphadenopathy or cutaneous sarcoid were mentioned. Cardiac, respiratory and neurological examinations were normal.[panafrican-med-journal.com]
Microscopic Hematuria
  • Moderate proteinuria was found in 31 (66%) patients (median, 0.7 g/24 h; range, 0-2.7 g/24 h), microscopic hematuria in 11 (21.7%) patients, aseptic leukocyturia in 13 (28.7%) patients. Fifteen of 47 (32%) patients had hypercalcemia ( 2.75 mmol/L).[ncbi.nlm.nih.gov]
  • Microscopic hematuria was present in one case. Renal failure and hypertension were absent. We did not notice any disorder of phosphate and calcium assesments.[ard.bmj.com]
Nocturia
  • A 66-year-old woman presented with a 1-year history of lethargy, polyuria and nocturia. Clinical examination was unremarkable and she had impaired renal function (urea 18 mmol/l (108 mg%) and creatinine 380 mumol/l (4.3 mg%)).[ncbi.nlm.nih.gov]
  • Patients frequently have nocturia (decreased concentrating ability), sterile pyuria, and HTN. Anaemia is frequently seen. 32.[slideshare.net]

Workup

  • Contralateral progression and workup for further symptoms finally established the diagnosis of extrapulmonary sarcoidosis. With corticosteroid and azathioprine treatment, renal function was preserved in the long term.[ncbi.nlm.nih.gov]
  • Extensive workup failed to yield a definitive diagnosis concerning the cause of the disorder. Intense uptake of 18 F-FDG in the spleen and liver was detected by PET/CT imaging with negative angiotensin-converting enzyme (ACE) in serum.[ncbi.nlm.nih.gov]
  • Because histopathological analysis of renal biopsy specimens can reveal various entities, a diagnostic workup is necessary in every patient with sarcoidosis.[journals.lww.com]
  • Workup for malignancy was negative including chest X-ray and bone marrow biopsy. He progressed into renal failure and ended up on dialysis.[avicennajmed.com]
  • The chronic kidney disease workup was negative. Imaging indicated nephromegaly and a normal CT of the chest. Bone marrow and kidney biopsies were obtained. The bone marrow biopsy showed extensive noncaseating granulomatous inflammation.[shmabstracts.com]
Soft Tissue Calcification
  • tissue calcification owing to sarcoidosis: the sunlight-cola connection , J Bone Miner Res , 2010 , vol. 25 (pg. 1695 - 1699 ) 22 Therapy for extrapulmonary sarcoidosis , Semin Respir Crit Care Med , 2002 , vol. 23 (pg. 589 - 596 ) 23 Prevention of corticosteroid-induced[academic.oup.com]
  • tissue calcification owing to sarcoidosis: the sunlight-cola connection, J Bone Miner Res, 2010 , vol. 25 (pg. 1695 - 1699 ) 22 Therapy for extrapulmonary sarcoidosis, Semin Respir Crit Care Med, 2002 , vol. 23 (pg. 589 - 596 ) 23 Prevention of corticosteroid-induced[academic.oup.com]
X-Ray Abnormal
  • Stages of sarcoidosis describe the chest x-ray abnormality: Stage I disease has only enlarged hilar and mediastinal lymph nodes (these gland sit outside the lungs); Stage II disease has enlarged lymph glands as seen in Stage I disease, but it also has[sarcoid-network.org]
Creatinine Decreased
  • After 2 months of prednisone therapy, serum creatinine decreased. However, because of continued of hypercalcemia unresponsive to low calcium diet and prednisone, chloroquine was prescribed.[ncbi.nlm.nih.gov]
Abnormal Renal Function
  • Case Report A 43-year-old African-American male with no prior medical history was referred to outpatient nephrology clinic for evaluation of abnormal renal function found on routine health exam. Serum creatinine level was 2.4 mg/dL.[ispub.com]

Treatment

  • Treatment with corticosteroids is successful in improving renal function, but relapse is common on steroid withdrawal and prolonged treatment is necessary for disease control.[ncbi.nlm.nih.gov]
  • Our treatment of this patient shows that treatment with MZR can allow steroid sparing and prevent recurrence in a patient with sarcoidosis.[ncbi.nlm.nih.gov]
  • We suggest that the key prognostic factor for renal survival in RS is the early response to treatment.[ncbi.nlm.nih.gov]
  • With corticosteroid and azathioprine treatment, renal function was preserved in the long term.[ncbi.nlm.nih.gov]
  • Abstract We herein report a case of renal sarcoidosis presenting as acute kidney injury (AKI) during treatment with etanercept for rheumatoid arthritis.[ncbi.nlm.nih.gov]

Prognosis

  • In the same way, familial sarcoidosis does not imply a worse prognosis, with several reported cases in whom treatment is not required but spontaneous remission happens.[erj.ersjournals.com]
  • Prognosis of Sarcoidosis and the Kidney The prognosis of this condition, like the natural history, is variable. The presence of scarring within the kidney carries a less favourable prognosis.[myvmc.com]
  • The prognosis of sarcoidosis is usually good with spontaneous remission but the chronic aspect can be seen in one third of cases and may be associated then with one or more organ dysfunctions [ 1, 7, 9, 10 ].[panafrican-med-journal.com]
  • Prognosis Mortality associated with sarcoidosis is estimated at between 0.5-5%.[orpha.net]

Etiology

  • Sarcoidosis is a systemic disease of unknown etiology that is characterized by chronic non-caseating granulomatous inflammation with tissue destruction.[ncbi.nlm.nih.gov]
  • Although uncommon, renal sarcoidosis should be considered in the differential diagnosis of acute or chronic renal failure of uncertain etiology, as early diagnosis and treatment can lead to recovery of renal function and prevent interstitial fibrosis.[ncbi.nlm.nih.gov]
  • Sarcoidosis is a systemic granulomatous disease of unknown etiology and is associated with a wide variety of renal disorders including nephrolithiasis, hypercalciuria, hypercalcemia, nephrocalcinosis, tubular defect, glomerulonephritis, and granulomatous[ncbi.nlm.nih.gov]
  • Introduction: Sarcoidosis is a systemic inflammatory disease of unknown etiology, characterized by the presence of non-caseating granulomas in several organs; renal impairment alone is a rare condition.[scielo.br]
  • Our experience indicates that the previously unreported etiology of acute renal failure in sarcoidosis, acute interstitial nephritis, can be successfully treated with prednisone. Author information Affiliations New York Univ.[nature.com]

Epidemiology

  • References [ 1 ] Löffler, U., Tuleweit, A., Waldherr, R., Uppenkamp, M. and Bergner, R. (2015) Renal Sarcoidosis: Epidemiological and Follow-Up Data in a Cohort of 27 Patients.[scirp.org]
  • Experts in the field provide chapters on epidemiology, genetics, pathology, ocular sarcoidosis, renal sarcoidosis, rare manifestations, diagnostic approaches, therapy, transplantation and more. Voransicht des Buches »[books.google.de]
  • Experts in the field provide chapters on epidemiology, genetics, pathology, ocular sarcoidosis, renal sarcoidosis, rare manifestations, diagnostic approaches, therapy, transplantation and more. Vista previa del libro »[books.google.es]
  • Renal Sarcoidosis: epidemiological and follow-up data in a cohort of 27 patients. Sarcoidosis Vasc Diffuse Lung Dis. 2015;31(4):306-15. PubMed Google Scholar Manjunath V, Moeckel G, Dahl NK.[panafrican-med-journal.com]
  • Renal involvement in sarcoidosis is much more common than generally assumed from old epidemiological studies and is often only detected when actively searched for. PubMed, Z Rheumatol, 04/12/2017.[sclero.org]
Sex distribution
Age distribution

Pathophysiology

  • Ackermann D (2007) Hypercalcemia in sarcoidosis–case report, prevalence, pathophysiology and therapeutic options.[link.springer.com]
  • Curr Opin Rheumatol 12 : 71 -76, 2000 Mentzer SJ: Dendritic cells in the pathophysiology of sarcoidal reactions.[jasn.asnjournals.org]
  • Physiologic and/or pathophysiologic implications Morbidity from sarcoidosis results from unchecked inflammation resulting in irreversible fibrosis and damage in affected organs.[renalandurologynews.com]
  • We discuss the atypical presentation, pathophysiology and highlight the importance of performing a renal biopsy.[ispub.com]
  • In this piece, we’ll briefly highlight facts regarding the epidemiology, pathophysiology, and diagnosis of sarcoidosis.[emdocs.net]

Prevention

  • Awareness of similarities in computed tomography imaging and histopathological findings of xanthogranulomatous pyelonephritis and renal sarcoidosis may prevent delayed diagnosis and inappropriate treatment of the latter.[ncbi.nlm.nih.gov]
  • TNF) therapy is an extremely rare manifestation, this case suggests that renal sarcoidosis should be considered in the differential diagnosis of AKI in patients receiving anti-TNF therapy, as providing an early diagnosis and treatment is important for preventing[ncbi.nlm.nih.gov]
  • Our treatment of this patient shows that treatment with MZR can allow steroid sparing and prevent recurrence in a patient with sarcoidosis.[ncbi.nlm.nih.gov]
  • Although uncommon, renal sarcoidosis should be considered in the differential diagnosis of acute or chronic renal failure of uncertain etiology, as early diagnosis and treatment can lead to recovery of renal function and prevent interstitial fibrosis.[ncbi.nlm.nih.gov]
  • High levels of calcium in the blood also cause blood vessels in the kidney to constrict, preventing proper clearance of waste from the blood and leading to dehydration .[sarcoidosisnews.com]

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