AML ASSOCIATED MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS

AML ASSOCIATED MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS

NKF 2015 Spring Clinical Meetings Abstracts 97 NEW DIAGNOSIS OF BULLOUS PEMPHIGOID AFTER WITHDRAWAL OF IMMUNOSUPPRESSIVE THERAPY IN A FAILED RENAL TR...

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NKF 2015 Spring Clinical Meetings Abstracts

97 NEW DIAGNOSIS OF BULLOUS PEMPHIGOID AFTER WITHDRAWAL OF IMMUNOSUPPRESSIVE THERAPY IN A FAILED RENAL TRANSPLANT RECIPIENT ON HEMODIALYSIS Torrance Green1, Amanda Quorles1, Kenneth D. Saul2, Jeremy D. Jackson2, Jack R. Lewin, Tibor Fülöp1. 1Department of Medicine and 2 Dermatology, University of Mississippi Medical Center, Jackson, MS, USA. During the course of end-stage renal disease (ESRD), the transition period during initiation of hemodialysis (HD) after a failed renal transplant (RTx) is associated with increased morbidity and mortality. A 44 year-old African-American female on chronic HD presented with 2-3 weeks of generalized aches and rash on her predominantly on her extremities, with involvement of her chest and flank. She reported no provoking trauma, associated pain, erythema or symptoms of infection. Comorbidities included insulin-dependent diabetes mellitus, hypertension and gout. She received a deceased-donor RTx which failed after 16 years (10 month prior to presentation) due to recurrent diabetic nephropathy; Mycophenolate mofetil was held at that time. Renal function continued to decline prompting transition to HD concurrent with tapering of immunosuppression (IS) therapy; tacrolimus and prednisone were held 2 and 1 month prior to presentation, respectively. Physical exam revealed scattered 3-8 mm erosions with few tense, 2-8 mm blisters. Initial differential included bullous pemphigoid, bullous lupus, pemphigus, linear IgA bullous dermatosis and herpes zoster. Punch skin biopsy revealed dermal-epidermal blister with associated mixed inflammation including eosinophils. Immunofluorescence studies confirmed 3+ linear fluorescence with anti-IgG and -C3 antibodies at the basement membrane, securing the diagnosis of bullous pemphigoid. IS therapy for RTx recipients is known to be active against bullous pemphigoid. Cessation of IS after a failed RTx may unmask an autoimmune disorder, such a bullous pemphigoid. Therefore, increased clinical suspicion is warranted during IS discontinuation and HD reinitiation.

98 PATIENT PERSPECTIVES ON CHRONIC KIDNEY DISEASE SELF-MANAGEMENT Jamie Green,1 Christina Yule,1 Andrea Berger,1 Steven Weisbord2; 1Geisinger Medical Center, Danville, PA; 2VA Pittsburgh Healthcare System, Pittsburgh, PA; USA. Little is known about patient self-management of chronic kidney disease (CKD). We sought to explore the perspectives of patients with CKD regarding factors they perceive as facilitators or barriers to effective self-management. We conducted a series of individual, telephone-based semistructured interviews in patients with non-dialysis dependent CKD stages 3B-5 followed at a single nephrology clinic in rural Pennsylvania. Patients were recruited as part of a larger study examining self-management behaviors and were stratified based on levels of health literacy and self-management skills. Patients were asked about their overall experience in managing their kidney health, what they find most difficult, what they find helpful, and what an ideal self-management program would look like. Interviews were audio recorded and transcribed verbatim for analysis. A total of 18 interviews were completed. Reported areas of difficulty included CKD knowledge, diet and fluid restrictions, managing medications, blood pressure control, interpretation of lab results, and dialysis decision-making. Patient utilized a variety of resources to help manage their kidney health including health professional assistance, support from family or friends, educational classes, or the Internet. Patients expressed interest in self-management programs that are longitudinal, delivered in plain language, offered via multiple modalities (in-person, online, print), and that include peer support. These findings can help inform the design of self-management interventions to improve CKD care.

Am J Kidney Dis. 2015;65(4):A1-A93

99 KIDNEY DISEASE IN HUMAN IMMUNODEFICIENCY VIRUS PATIENTS IS NOT ALWAYS HIV-ASSOCIATED NEPHROPATHY (HIVAN), BUT HIV IMMUNE COMPLEX KIDNEY DISEASE (HIVICK) AS WELL Ujwala Gunnal, Alison Ulbrant, Avantika Chenna, Punita Kaveti, Chamberlain Obialo, Khalid Bashir, Morehouse School of Medicine, Atlanta, GA. Kidney disease in Human Immunodeficiency (HIV) patients is most commonly attributed to HIVAN. Prevalence of kidney disease in HIV patients is 2.4 % to 12 %. In addition to HIVAN, HIVICK is being more recognized in HIV patients. We present a case of 55 yo female with biopsy confirmed HIVICK. 55 yo F with PMH of HIV not on ART, Hypertension, Chronic kidney disease stage 3 presented with complaints of four months of worsening leg swelling and decreased urine output. Vitals were significant for blood pressure of 150/90. Physical exam showed pallor and 2+ pitting edema of legs. Initial labs showed BUN of 25 mg/dl, Creatinine of 2.1 mg/dl, Hemoglobin of 6.1 g/dl, CH50 was 28U/ml, SPEP was negative, CD4 count was 100/mcl. Urinalysis showed 3+ protein and Urine Protein/Creatinine ratio was 8g/day. There was high suspicion for HIVAN but CT guided biopsy showed histologic findings consistent with HIVICK and HIVAN. She was managed with diuretics, lisinopril and HAART therapy with improvement in symptoms. HIVICK is a term given to pathologic findings consistent with “lupus-like” GN, IgA Nephropathy, PIGN and Immune Complex disease not otherwise specified. HIVICK is commonly seen in African Americans, associated with severe HIV, Hypertension and unlike HIVAN less likely to progress to ESRD. It is characterized by classic finding of “ball in cup” basement membrane lesion on biopsy. Viral replication or immune responses to viral proteins are essential to trigger HIVICK. Very few cases of HIVICK have been reported. Histopathology of the kidney is crucial for early diagnosis, prognosis and treatment of this disorder in an effort to prevent further morbidity.

100 AML ASSOCIATED MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS Sagar Gupta, Timothy Yau, Sambhavi Krishnamoorthy, Washington University, Saint Louis, Missouri, USA Membranoproliferative glomerulonephritis (MPGN) as a paraneoplastic glomerulopathy has been previously reported in association with lung, gastrointestinal and urogenital tumors. We describe a case of biopsy proven MPGN associated with Acute Myeloblastic Leukemia (AML), an extremely rare entity. A 62 year old Caucasian male was admitted to the hospital after being diagnosed with AML (type M6a). He was started on induction chemotherapy with 7+3 cytarabine and idarubicin. One week later, he started developing anasarca, decreasing urine output and shortness of breath. Lab testing revealed acute kidney injury with rise of serum creatinine to 4.57 mg/dL from a baseline of 0.66. He had nephrotic range proteinuria with 6.9 grams spot urine protein/creatinine ratio. Additional infectious and rheuamatological workup was negative for ANA, ANCA, antiGBM Ab, cyroglobulins, HIV, Hepatitis B and C and normal serum complements. He was started on hemodialysis for worsening fluid overload and azotemia. Renal biopsy was performed and light microscopy showed non-sclerosed glomeruli with mesangial hypercellularity (neutrophilic infiltrates) and thickened capillary loops. Immunofluorescence was positive for IgG, IgA, IgM and C3 while electron microscopy revealed dense subendothelial deposits and no cyroglobulins. This was consistent with a diagnosis of secondary MPGN. He was started on high-dose corticosteroids, diuretics and ACE inhibitor. There was dramatic improvement in symptoms with complete resolution of renal dysfunction and proteinuria. While paraneoplastic glomerulonephritides (especially Minimal Change Disease) are frequently associated with lymphoid malignancies, they are exceedingly rare with acute myeloid neoplasms. Blast cells producing nephrotoxic cytokines and immune complex mediated renal injury have been proposed in their pathogenesis. Trial of corticosteroids and chemotherapy against primary cancer remain the mainstay of treatment.

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