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IGA-DOMINANT STAPHYLOCOCCUS INFECTION ASSOCIATED GLOMERULONEPHRITIS WITH HENOCH-SCHONLEIN PURPURA LIKE PRESENTATION
NKF 2015 Spring Clinical Meetings Abstracts
289 IGA-DOMINANT STAPHYLOCOCCUS INFECTION ASSOCIATED GLOMERULONEPHRITIS WITH HENOCH-SCHONLEIN PURPURA LIKE PRESENTATION Narittaya Varothai, Sindhu Arjun. Tufts Medical Center, Boston, MA, USA Henoch-Schonlein purpura (HSP) is caused by small-vessel vasculitis associated with IgA-containing immune complex deposits causing purpuric skin rash, IgA dominant glomerulonephritis (GN), and joint pain. A similar presentation can be seen in both primary IgA disease and systemic infection with Staphylococcus Aureus. It is important to differentiate between the two conditions to avoid mistreatment. Case: A 72 year old male developed empyema, knee swelling, and rash 3 months after pneumonia; pleural fluid cultures grew MRSA. Skin biopsy showed leukoclastic vasculitis. Creatinine increased to 1.23 mg/dL, urine sediment showed cellular casts, urine PCR was 2 g/g. Serological workup was negative. Linezolid was started but creatinine and proteinuria worsened. Kidney biopsy showed crescentic glomerulonephritis with glomerular IgA deposits suspicious for Staphylococcus associated IgA mediated GN. Pulse dose steroids were given. After steroid taper and antibiotics, infection was eradicated and creatinine stabilized at 1.7 to 1.9 mg/dl. IgA-dominant Staphylococcus associated GN is mediated by superantigens and an IgA-immune response from mucosal infections. It is impossible to distinguish between primary IgA and staphylococcus associated IgA GN based on biopsy alone. Characteristics such as age, active infection, and time of presentation can prove to be clues. Staph associated GN is treated with antibiotics, while primary IgA GN is treated with immunosuppressants. Steroids can be considered in some cases. In our patient, concomitant therapy with steroids helped stabilize renal function.
290 PARTIAL REMISSION OF FIBRILLLARY GLOMERULONEPHRITIS WITH CRESCENTIC AND NECROTIZING FEATURES TREATED WITH H.P. ACTHAR® GEL Dmitri Vasin, Renal Remission and Hypertension Consultants PLLC, Silverdale, WA, USA Fibrillary glomerulonephritis (GN) is an unusual cause of rapidly progressive necrotizing crescenting GN. This is a case of a 67-year-old female presenting with pulmonary renal syndrome, microhematuria, nephrotic proteinuria (11.8 g/g), and advanced renal failure (serum creatinine [SCr] of 12 mg/dl). Kidney biopsy showed necrotizing crescentic GN, involving 80100% of glomeruli without vasculitis and with linear staining of glomerular basement membrane (GBM) for IgG and C3 on immunofluorescence. Negative results of anti-GBM antibody and finding of pathognomonic fibrillary deposits on electron microscopy and resolution of pulmonary infiltrates with ultrafiltration on hemodialysis (HD) established diagnosis of fibrillary GN. Initial therapy with pulse steroids, plasmapheresis, cyclophosphamide, and prednisone was stopped. Patient remained HD dependent, anuric, with SCr 8.8-9.6 mg/dl. After 4 weeks with no GN specific therapy, H.P. Acthar® Gel (repository corticotropin injection, Questcor Pharmaceuticals, Inc., Hayward, CA), 8 U sc QD was started and increased to 16 U sc QD after 4 weeks. Rapid reversal of anuria, with no interdialytic weight gain ensued. No loop diuretics were used. Spot urine albumin-to-creatinine ratio decreased from 5026 to 1875 mg/g. Improvement in renal function (SCr 5.56 mg/dl) was observed. No side effects of therapy besides mild cushingoid features noted. After 8 weeks of therapy with H.P. Acthar® Gel partial remission of fibrillary GN was achieved. Editorial assistance provided by MedVal Scientific Information Services, LLC supported by Mallinckrodt Pharmaceuticals.
Am J Kidney Dis. 2015;65(4):A1-A93
291 HYPERNATREMIA: A SURROGATE MARKER FOR SEPSIS? Myriam Vela-Ortiz, Tun Nay Thi, Jacek Jodelka, David Livert, Mahesh Krishnamurthy, Richard Snyder. Easton Hospital, Drexel College of Medicine, Easton, PA , USA Introduction: Hypernatremia is an extremely common condition observed in elderly patients upon hospital admission. The mortality rate of patients with hypernatremia is significantly higher than that of “eunatremic” patients. Methods: We evaluated retrospectively the records from 2008 to 2010 of patients with high sodium levels on admission, greater than 145 mmmol/L. Patients who received a diagnosis of sepsis or SIRS as documented either on the admission note or discharge summary were included in the cohort. Results: We reviewed the medical records of 150 patients older than 60 years old who on admission had hypernatremia. 77.4% also were diagnosed with infection, SIRS or sepsis on admission and 87.7% at the time of discharge. The mean age for the group was 82 years. The most common infection type associated with hypernatremia was Urinary Tract Infection (UTI) (46.3%) followed by pneumonia (41.3%). Hypertension was the most prevalent comorbidity (50%), followed by CAD (30%). 50 % of the patients had a sodium on admission in between 145 and 155 mmol/L and 10 % had a sodium level higher than 165mmol/L. Hypokalemia was concomitantly present in 22% of the patients, hyperglycemia in 40 % of the patients and elevated creatinine in 54% of the patients. Conclusions: Our study shows a positive correlation between hypernatremia found on admission and the presence of concomitant infection especially in the elderly population and in patients who carried a diagnosis of dementia in whom the thirst center may be altered. We suggest that the existence of hypernatremia should spur the clinician to further investigate for a possible source of infection especially in elderly patients.
292 NEPHROTIC SYNDROME AND POLYCYTHEMIA Vela-Ortiz Myriam, Hazlett Shawn , Reichart James , Easton Hospital, Internal Medicine, Nephrology Department, Easton, PA 18042 Case Description: An 18 -year -old male, who was treated twice as a child for minimal change disease, presented to our hospital with nephrotic syndrome. His first episode was at age 2 when he was treated with chlorambucil and then at age 17 when he was treated with steroids alone. During these two episodes the patient responded well to empiric therapy and did not require biopsy. Other past medical history included bipolar disorder and occasional marijuana use. His medications were Lithium and Risperdal. He presented to the hospital with generalized swelling, back pain, nausea, abdominal pain, and mild shortness of breath with normal oxygen saturation. His hemoglobin on admission was 20.6 g/dl, hematocrit of 61 %, white count 12.3 k/ul , platelet count 283000 k/ul , sodium 133 mmol/l, potassium 4.8 mEq/L, creatinine 0.7 mg/dl. Urinalysis showed proteinuria of 4 +, 50 to 100/hpf WBC, and 3- 5/ hpf RBC. Imaging studies included Doppler of the lower extremities, which was negative Normal kidney ultrasound with trace ascites. Initial treatment included Lasix and urgent phlebotomy with resolution of his dyspnea. Empiric steroid treatment was started for minimal change disease and he responded well over the next several weeks with resolution of his edema and proteinuria. Additional hematological work up showed negativity for JAK 2 mutation and erythropoietin level of 5. His hematocrit on follow up was less than 40 % on several occasions and he required no further phlebotomy. Discussion: This is a unique case of recurrent nephrotic syndrome with associated polycythemia. Interestingly, the patient’s two previous presentations were not associated with polycythemia. Given the absence of JAK 2 mutation, polycythemia vera was unlikely. The normal erythropoietin (EPO) in this patient made hypoxia a less likely etiology for the erythrocytosis. His polycythemia could also been compounded by third spacing and relative volume depletion. The etiology for the polycythemia in nephrotic syndrome cases seems to be related to higher EPO levels, increased sensitivity to EPO. Though it seems that the resolution of the nephrotic syndrome also alleviates the erythrocytosis as presented in our case.
A87
289 IGA-DOMINANT STAPHYLOCOCCUS INFECTION ASSOCIATED GLOMERULONEPHRITIS WITH HENOCH-SCHONLEIN PURPURA LIKE PRESENTATION Narittaya Varothai, Sindhu Arjun. Tufts Medical Center, Boston, MA, USA Henoch-Schonlein purpura (HSP) is caused by small-vessel vasculitis associated with IgA-containing immune complex deposits causing purpuric skin rash, IgA dominant glomerulonephritis (GN), and joint pain. A similar presentation can be seen in both primary IgA disease and systemic infection with Staphylococcus Aureus. It is important to differentiate between the two conditions to avoid mistreatment. Case: A 72 year old male developed empyema, knee swelling, and rash 3 months after pneumonia; pleural fluid cultures grew MRSA. Skin biopsy showed leukoclastic vasculitis. Creatinine increased to 1.23 mg/dL, urine sediment showed cellular casts, urine PCR was 2 g/g. Serological workup was negative. Linezolid was started but creatinine and proteinuria worsened. Kidney biopsy showed crescentic glomerulonephritis with glomerular IgA deposits suspicious for Staphylococcus associated IgA mediated GN. Pulse dose steroids were given. After steroid taper and antibiotics, infection was eradicated and creatinine stabilized at 1.7 to 1.9 mg/dl. IgA-dominant Staphylococcus associated GN is mediated by superantigens and an IgA-immune response from mucosal infections. It is impossible to distinguish between primary IgA and staphylococcus associated IgA GN based on biopsy alone. Characteristics such as age, active infection, and time of presentation can prove to be clues. Staph associated GN is treated with antibiotics, while primary IgA GN is treated with immunosuppressants. Steroids can be considered in some cases. In our patient, concomitant therapy with steroids helped stabilize renal function.
290 PARTIAL REMISSION OF FIBRILLLARY GLOMERULONEPHRITIS WITH CRESCENTIC AND NECROTIZING FEATURES TREATED WITH H.P. ACTHAR® GEL Dmitri Vasin, Renal Remission and Hypertension Consultants PLLC, Silverdale, WA, USA Fibrillary glomerulonephritis (GN) is an unusual cause of rapidly progressive necrotizing crescenting GN. This is a case of a 67-year-old female presenting with pulmonary renal syndrome, microhematuria, nephrotic proteinuria (11.8 g/g), and advanced renal failure (serum creatinine [SCr] of 12 mg/dl). Kidney biopsy showed necrotizing crescentic GN, involving 80100% of glomeruli without vasculitis and with linear staining of glomerular basement membrane (GBM) for IgG and C3 on immunofluorescence. Negative results of anti-GBM antibody and finding of pathognomonic fibrillary deposits on electron microscopy and resolution of pulmonary infiltrates with ultrafiltration on hemodialysis (HD) established diagnosis of fibrillary GN. Initial therapy with pulse steroids, plasmapheresis, cyclophosphamide, and prednisone was stopped. Patient remained HD dependent, anuric, with SCr 8.8-9.6 mg/dl. After 4 weeks with no GN specific therapy, H.P. Acthar® Gel (repository corticotropin injection, Questcor Pharmaceuticals, Inc., Hayward, CA), 8 U sc QD was started and increased to 16 U sc QD after 4 weeks. Rapid reversal of anuria, with no interdialytic weight gain ensued. No loop diuretics were used. Spot urine albumin-to-creatinine ratio decreased from 5026 to 1875 mg/g. Improvement in renal function (SCr 5.56 mg/dl) was observed. No side effects of therapy besides mild cushingoid features noted. After 8 weeks of therapy with H.P. Acthar® Gel partial remission of fibrillary GN was achieved. Editorial assistance provided by MedVal Scientific Information Services, LLC supported by Mallinckrodt Pharmaceuticals.
Am J Kidney Dis. 2015;65(4):A1-A93
291 HYPERNATREMIA: A SURROGATE MARKER FOR SEPSIS? Myriam Vela-Ortiz, Tun Nay Thi, Jacek Jodelka, David Livert, Mahesh Krishnamurthy, Richard Snyder. Easton Hospital, Drexel College of Medicine, Easton, PA , USA Introduction: Hypernatremia is an extremely common condition observed in elderly patients upon hospital admission. The mortality rate of patients with hypernatremia is significantly higher than that of “eunatremic” patients. Methods: We evaluated retrospectively the records from 2008 to 2010 of patients with high sodium levels on admission, greater than 145 mmmol/L. Patients who received a diagnosis of sepsis or SIRS as documented either on the admission note or discharge summary were included in the cohort. Results: We reviewed the medical records of 150 patients older than 60 years old who on admission had hypernatremia. 77.4% also were diagnosed with infection, SIRS or sepsis on admission and 87.7% at the time of discharge. The mean age for the group was 82 years. The most common infection type associated with hypernatremia was Urinary Tract Infection (UTI) (46.3%) followed by pneumonia (41.3%). Hypertension was the most prevalent comorbidity (50%), followed by CAD (30%). 50 % of the patients had a sodium on admission in between 145 and 155 mmol/L and 10 % had a sodium level higher than 165mmol/L. Hypokalemia was concomitantly present in 22% of the patients, hyperglycemia in 40 % of the patients and elevated creatinine in 54% of the patients. Conclusions: Our study shows a positive correlation between hypernatremia found on admission and the presence of concomitant infection especially in the elderly population and in patients who carried a diagnosis of dementia in whom the thirst center may be altered. We suggest that the existence of hypernatremia should spur the clinician to further investigate for a possible source of infection especially in elderly patients.
292 NEPHROTIC SYNDROME AND POLYCYTHEMIA Vela-Ortiz Myriam, Hazlett Shawn , Reichart James , Easton Hospital, Internal Medicine, Nephrology Department, Easton, PA 18042 Case Description: An 18 -year -old male, who was treated twice as a child for minimal change disease, presented to our hospital with nephrotic syndrome. His first episode was at age 2 when he was treated with chlorambucil and then at age 17 when he was treated with steroids alone. During these two episodes the patient responded well to empiric therapy and did not require biopsy. Other past medical history included bipolar disorder and occasional marijuana use. His medications were Lithium and Risperdal. He presented to the hospital with generalized swelling, back pain, nausea, abdominal pain, and mild shortness of breath with normal oxygen saturation. His hemoglobin on admission was 20.6 g/dl, hematocrit of 61 %, white count 12.3 k/ul , platelet count 283000 k/ul , sodium 133 mmol/l, potassium 4.8 mEq/L, creatinine 0.7 mg/dl. Urinalysis showed proteinuria of 4 +, 50 to 100/hpf WBC, and 3- 5/ hpf RBC. Imaging studies included Doppler of the lower extremities, which was negative Normal kidney ultrasound with trace ascites. Initial treatment included Lasix and urgent phlebotomy with resolution of his dyspnea. Empiric steroid treatment was started for minimal change disease and he responded well over the next several weeks with resolution of his edema and proteinuria. Additional hematological work up showed negativity for JAK 2 mutation and erythropoietin level of 5. His hematocrit on follow up was less than 40 % on several occasions and he required no further phlebotomy. Discussion: This is a unique case of recurrent nephrotic syndrome with associated polycythemia. Interestingly, the patient’s two previous presentations were not associated with polycythemia. Given the absence of JAK 2 mutation, polycythemia vera was unlikely. The normal erythropoietin (EPO) in this patient made hypoxia a less likely etiology for the erythrocytosis. His polycythemia could also been compounded by third spacing and relative volume depletion. The etiology for the polycythemia in nephrotic syndrome cases seems to be related to higher EPO levels, increased sensitivity to EPO. Though it seems that the resolution of the nephrotic syndrome also alleviates the erythrocytosis as presented in our case.
A87