Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

AL amyloidosis is due to deposition of protein derived from immunoglobulin light chain fragments. It is a systemic disorder in which deposition of plasma proteins can adversely affect function of the heart, liver, kidneys, and peripheral nerves. Deposition in the heart results in a decrease in the amplitude of the electrical activity of the heart and can be an early clue to the diagnosis. A 63-year-old male admitted for volume overload was found to have nephrotic range proteinuria, progressive renal insufficiency (Creatinine 4.0 increased from his baseline 0.9), and hypoalbuminemia. On exam, he had diffuse anasarca and peripheral neuropathy. A renal biopsy showed AL amyloidosis, lambda related, involving the glomeruli, interstitium, and arterial walls. Bone marrow biopsy showed 30% plasma cells with lambda light chain predominance. Serum free light chains were elevated. Lamda was 11.50 mg/dL and kappa was 5.12 mg/dL. In retrospective review of his chart, an EKG with low voltage and anterior pseudo-infarct pattern was first apparent on an admission for stroke two years prior. Echocardiogram showed mild concentric left ventricular hypertrophy. The patient was started on chemotherapy with Bortezomib. The differential of a low-voltage EKG includes many common pulmonary and chest wall (COPD, obesity) as well as pericardial diseases (effusions), but also important rarer infiltrative diseases including sarcoidosis and amyloidosis. Amyloidosis of the heart can cause progressive irreversible heart failure, but its progress can be altered if identified early. Physicians should consider amyloidosis when faced with a low-voltage EKG along with systemic symptoms including nephrotic range proteinuria, peripheral neuropathy, hepatosplenomegaly, and macroglossia.
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PMID:Low voltage criteria EKG as a harbinger of systemic disease. 3125 62

Sarcoidosis is a multisystem granulomatous disease of unknown etiology that can present with nonspecific features, often resulting in delayed diagnosis. The diagnosis requires the demonstration of non-caseating granulomas on biopsy. While the prevalence of sarcoidosis in the USA is rare, the disease is rarer yet in Hispanics. It is for this reason that we report herein the case of a Hispanic gentleman with a unique clinical manifestations of sarcoidosis. With what began as a two-month history of joint pain and skin rash, this 55-year-old man was hospitalized with multiple joint pain, weight loss, fatigue and a pruritic rash with leonine facies in the setting of anemia, leukopenia, hypercalcemia, elevated serum creatinine, and urine Bence-Jones proteinuria. CT imaging of the chest was nonspecific, but skin biopsy revealed non-caseating granulomatous disease. After completing an infectious and malignancy evaluation, the patient was diagnosed with sarcoidosis, which was treated successfully with low-dose steroid therapy.
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PMID:Atypical manifestations of sarcoidosis in a Hispanic male. 3125 73

Sarcoidosis is a systemic disorder affecting multiple organs. We presented a 56-year-old woman with renal impairment who was diagnosed with sarcoidosis accompanied by IgA nephropathy. Treatment with methylprednisolone was started for the patient. After treatment, the patient was discharged with good general condition and resolved proteinuria.
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PMID:Acute renal failure due to IgA nephropathy in sarcoidosis. 3170 52

Renal involvement is rare in systemic sarcoidosis. Among renal manifestations, tubulointerstitial nephritis (TIN) is the most commonly reported finding. We conducted the current study to investigate the clinical, laboratory, and histological features and to analyze the outcome of TIN due to sarcoidosis. We present a retrospective, single-center study of patients followed for sarcoidosis and presenting with TIN related to this systemic disease. Twenty-four patients were assessed (22 females/2 males). The mean age at diagnosis of TIN was 46.3 years. Extrarenal manifestations were dominated by thoracic involvement (95.8%), peripheral lymph nodes (54.2%), and skin lesions (33.3%). The mean proteinuria level was 0.68 g/24 h. Renal failure was diagnosed in 83.3% of cases with a median estimated glomerular filtration rate at 14.3 mL/min/1.73 m2. Nine patients presented with hypercalcemia and 12 patients with hypercalciuria. Renal biopsy was performed in 58.3% of cases. Six of the 14 patients presented with noncaseating granulomatous interstitial nephritis and eight with interstitial nephritis without granuloma. Granulomatous infiltration of renal parenchyma was complicated by vasculitis in two cases. Corticosteroid therapy was used in all patients. On follow-up analysis, four patients progressed to end-stage renal disease (ESRD) after a mean duration at 45.5 months. In the remaining patients, kidney function statistically significantly improved after one month of treatment compared to the time when the diagnosis was initially established (P = 0.031). We found that the predictive factors of progression to ESRD were multiorgan involvement (P = 0.032), advanced fibrosis F3 (P = 0.0006), and extensive interstitial granulomas (P = 0.007) and these were independently correlated with ESRD. Corticosteroid therapy seems to be effective in sarcoid TIN, but some degree of persistent renal failure is possible which can be predicted from both histologic findings and initial response to steroid therapy.
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PMID:Tubulointerstitial nephritis due to sarcoidosis: Clinical, laboratory, and histological features and outcome in a cohort of 24 patients. 3192 74

Introduction: While sarcoidosis has been recognized as a potential cause of proteinuria, no study has systematically evaluated the prevalence and risk factors for proteinuria in sarcoid patients. Methods: Consecutive sarcoid patients followed in a university clinic were identified prospectively. All patients with spot urine protein-to-creatinine ratio (UPCR) between 11-2012 and 07-2015 were included in the analysis. Proteinuria was defined as a spot UPCR equal to or exceeding 0.3 mg/mg. The primary goal of the study was to determine the prevalence of proteinuria in this sarcoidosis cohort. Results: Our study cohort consisted of 190 sarcoidosis patients (65% female, 82% white, mean age of 53 years (range 24-88)). Proteinuria was present in 14/190 (7%) of this cohort. Only5/190 patients (2.5%) had proteinuria who did not have a risk factor for proteinuria. Estimating the 24-hour urine protein excretion by extrapolating from the spot UPCR, proteinuria was moderate in amount (mean 1.60, range 0.32-5.06 mg/mg). Proteinuric patients received a lower mean daily dose of corticosteroids compared to those without proteinuria (0 mg vs 4.7 mg of prednisone); however, this difference did not reach statistical significance (p = 0.20). Conclusion: Our study found proteinuria in 7% of the 190 sarcoid patients. More than half of the patients with proteinuria had a known risk factor for proteinuria other than sarcoidosis. Proteinuria is uncommon in sarcoidosis, and, when it occurs, it should not be assumed that sarcoidosis is the cause without investigating alternative causes of proteinuria. (Sarcoidosis Vasc Diffuse Lung Dis 2017; 34: 142-148).
Sarcoidosis Vasc Diffuse Lung Dis 2017
PMID:Proteinuria in sarcoidosis: Prevalence and risk factors in a consecutive outpatient cohort. 3247 35

We experienced a rare case of tubulointerstitial angiocentric granulomatous vasculitis with focal segmental glomerulosclerosis (FSGS) and associated sarcoidosis. Our patient was an 18-year-old man who presented with exertional cough and dyspnea. He also had overt proteinuria (3.0 g/24 h), normal renal function (eGFR 95 mL/min/1.73 m<sup>2</sup>), heart failure, and hypertension. He had no previous episode of hypertension. These manifestations immediately improved after the administration of antihypertensive therapy that contained an angiotensin-converting enzyme inhibitor, calcium antagonists, beta antagonists, and diuretics. However, he, later on, developed renal dysfunction, with worsening of both proteinuria and hypertension. Renal biopsy was performed and showed epithelioid cells that were arranged concentrically around small blood vessels in tubulointerstitial granulomas. In the glomeruli, the segmental sclerotic lesions were classified as a perihilar variant of FSGS. There were no inflammatory changes, such as a mesangial lesion, inflammatory cell infiltration, fibrinoid necrosis, or crescent formation, and no glomerular granuloma. In the tubulointerstitial granulomas, the intimal elastic lamina of the interlobular arteries was reduplicated, and the intimal wall thickness of renal arterioles was remarkable. After receiving oral prednisolone therapy, the overt proteinuria resolved, the eGFR recovered from 39.4 to 60.6 mL/min/1.73 m<sup>2</sup>, and hypertension was managed more easily. Thereafter, he did not experience any recurrence. The concurrent improvement of renal function and proteinuria by steroid treatment suggested a relationship between the glomerular lesions and the tubulointerstitial granulomatous vasculitis with associated sarcoidosis.
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PMID:A Case Demonstrating the Pathological Relationship between Granulomatous Vasculitis and Glomerular Lesion in Renal Sarcoidosis. 3317 84


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