Dataset Information

Antibody-mediated rejection 16 years post-cardiac transplantation: a case report of an uncommon late presentation in a middle-aged woman.

ABSTRACT:

Background?

Very late antibody-mediated rejection (AMR) in heart transplant patients (over 10?years post-transplant) is very rare. It is associated with high mortality, graft dysfunction, and fulminant coronary artery vasculopathy (CAV) and should remain in the differential for patients presenting with late graft dysfunction.

Case summary?

A 57-year-old woman 16?years of post-heart transplant with a previously unremarkable post-transplant course including protocol driven biopsies showing no rejection and a recent unremarkable screening nuclear stress test presented to our institution with clinical heart failure. Echocardiogram revealed graft dysfunction and endomyocardial biopsy showed no signs of cellular rejection, but evidence of AMR. The patient was treated with steroid and immunotherapy with clinical improvement but suffered several infectious complications and renal dysfunction requiring haemodialysis related to her immunotherapy treatment. Despite aggressive AMR management, donor-specific antibodies and symptoms persisted and CAV progressed.

Discussion?

This case illustrates the poor diagnostic yield of non-invasive testing for AMR, and highlights importance to clinicians of considering AMR even if the patient over 10?years post-transplant when the diagnosis is rare.

SUBMITTER: Miller C

PROVIDER: S-EPMC6764549 | biostudies-literature | 2019 Sep

REPOSITORIES: biostudies-literature

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Publications

Antibody-mediated rejection 16 years post-cardiac transplantation: a case report of an uncommon late presentation in a middle-aged woman.

Miller Charles C Arkun Knarik K DeNofrio David D Sabe Marwa M

European heart journal. Case reports 20190901 3

<h4>Background</h4>Very late antibody-mediated rejection (AMR) in heart transplant patients (over 10 years post-transplant) is very rare. It is associated with high mortality, graft dysfunction, and fulminant coronary artery vasculopathy (CAV) and should remain in the differential for patients presenting with late graft dysfunction.<h4>Case summary</h4>A 57-year-old woman 16 years of post-heart transplant with a previously unremarkable post-transplant course including protocol driven biopsies sh ...[more]

PMID: 31365068

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Project description:ImportanceAcute rejection is one of the most frequent complications in facial transplantation, with potentially severe consequences for the recipient if overlooked. Clinical signs, such as erythema or edema, are helpful to diagnose acute rejection in the early follow-up stage; however, it is not well known whether these clinical signs remain reliable markers of acute rejection beyond the second posttransplant year.ObjectiveTo determine the diagnostic value of clinical signs of acute rejection after facial transplantation over time.Design, setting, and participantsA retrospective, single-center cohort study was conducted of patients who underwent facial transplantation at Brigham and Women's Hospital between April 2009 and October 2014, with up to an 8-year follow-up. Medical records were reviewed until September 30, 2017. The medical records from 104 encounters with 7 patients who underwent partial or full facial transplantation were analyzed for symptoms of rejection, immunosuppressive therapy, and histopathologic findings.Main outcomes and measuresThe occurrence of 5 clinical signs of acute rejection were evaluated: erythema, edema, exanthema, suture line erythema, and mucosal lesions. Odds ratios (ORs) were calculated to determine the statistically significant association of these signs with the histopathologic diagnosis of rejection. In addition, tacrolimus blood levels, as a surrogate marker of immunosuppressive therapy, were evaluated.ResultsOf the 7 patients included in the study, 5 were men. The mean follow-up was 66 months (range, 35-101). Of 104 clinical encounters, 46 encounters (44.2%) represented rejection episodes and 58 encounters (55.8%) represented no-rejection episodes. Beyond 2 years posttransplantation, only erythema (OR, 6.53; 95% CI, 1.84-20.11; P = .004) and exanthema (OR, ∞; 95% CI, 2.2-∞; P = .004) were demonstrated to be reliable clinical signs of acute rejection in facial transplantation. There was also a statistically significant association of subtherapeutic tacrolimus levels with late rejection episodes (OR, 3.79; 95% CI, 1.25-12.88; P = .03). In addition, the occurrence of subclinical rejection was more frequent during later follow-up times (7 [24.1%] late rejections vs 1 [5.9%] early rejection). Five of 8 subclinical rejections (62.5%) were associated with subtherapeutic tacrolimus levels.Conclusions and relevanceClinical signs of acute rejection in facial transplantation appear to be of limited diagnostic value, particularly after the second postoperative year. Until alternative biomarkers for rejection are identified, protocol skin biopsies will remain necessary for guiding assessments of allograft rejection.Level of evidence3.

Project description:Widely distributed osteosclerosis is an unusual radiographic finding with multiple causes. A 42-year-old premenopausal Spanish woman gradually acquired dense bone diffusely affecting her axial skeleton and focally affecting her proximal long bones. Systemic lupus erythematosus (SLE) diagnosed in adolescence had been well controlled. She had not fractured or received antiresorptive therapy, and she was hepatitis C virus antibody negative. Family members had low bone mass. Lumbar spine bone mineral density (BMD) measured by dual-photon absorptiometry (DPA) at age 17 years, while receiving glucocorticoids, was 79% the average value of age-matched controls. From ages 30 to 37 years, dual-energy X-ray absorptiometry (DXA) BMD Z-scores steadily increased in her lumbar spine from +3.8 to +7.9, and in her femoral neck from -1.4 to -0.7. Serum calcium and phosphorus levels were consistently normal, 25-hydroxyvitamin D (25OHD) <20?ng/mL, and parathyroid hormone (PTH) sometimes slightly increased. Her reduced estimated glomerular filtration rate (eGFR) was 38 to 55?mL/min. Hypocalciuria likely reflected positive mineral balance. During increasing BMD, turnover markers (serum bone-specific alkaline phosphatase [ALP], procollagen type 1?N propeptide [P1NP], osteocalcin [OCN], and carboxy-terminal cross-linking telopeptide of type 1 collagen [CTx], and urinary amino-terminal cross-linking telopeptide of type 1 collagen [NTx and CTx]) were 1.6- to 2.8-fold above the reference limits. Those of bone formation seemed increased more than those of resorption. FGF-23 was slightly elevated, perhaps from kidney disease. Serum osteoprotegerin (OPG) and TGF?1 levels were normal, but sclerostin (SOST) and receptor activator of nuclear factor kappa-B ligand (RANKL) were elevated. Serum multiplex biomarker profiling confirmed a high level of SOST and RANKL, whereas Dickkopf-1 (DKK-1) seemed low. Matrix metalloproteinases-3 (MMP-3) and -7 (MMP-7) were elevated. Iliac crest biopsy revealed tetracycline labels, no distinction between thick trabeculae and cortical bone, absence of peritrabecular fibrosis, few osteoclasts, and no mastocytosis. Then, for the past 3 years, BMD Z-scores steadily decreased. Skeletal fluorosis, mastocytosis, myelofibrosis, hepatitis C-associated osteosclerosis, multiple myeloma, and aberrant phosphate homeostasis did not explain her osteosclerosis. Mutation analysis of the LRP5, LRP4, SOST, and osteopetrosis genes was negative. Microarray showed no notable copy number variation. Perhaps her osteosclerosis reflected an interval of autoimmune-mediated resistance to SOST and/or RANKL. © 2016 American Society for Bone and Mineral Research.

Dataset Information

Antibody-mediated rejection 16 years post-cardiac transplantation: a case report of an uncommon late presentation in a middle-aged woman.

Background?

Case summary?

Discussion?

Publications

Antibody-mediated rejection 16 years post-cardiac transplantation: a case report of an uncommon late presentation in a middle-aged woman.

Similar Datasets

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