Acute Kidney Injury in Systemic Sclerosis Beyond Scleroderma Renal Crisis: A Case-Control Study.

BACKGROUND AND OBJECTIVES: Renal involvement in systemic sclerosis remains a significant concern with the focus often centered on scleroderma renal crisis (SRC). However, the broader spectrum of renal manifestations, beyond SRC, remains underrecognized. In our case-control analysis, we describe other causes, risk factors, and renal outcomes of acute kidney injury (AKI) in systemic sclerosis other than SRC. METHODS: Patients diagnosed with SSC, with and without AKI, between 2017 and 2023 at Albany Medical Center, were included in the case-control study using International Classification of Diseases, 10th Revision codes and electronic medical records. Patients with SRC were carefully excluded. Data were collected and compared between AKI and non-AKI groups for patients' demographics, clinical characteristics, and baseline treatment. Additionally, data were collected for baseline, peak, and follow-up creatinine, etiology of AKI, treatment, and outcomes. Statistical analysis was performed using R (version 4.3.0) and Minitab (V19). Categorical variables were presented as frequencies/percentages, and continuous variables as means/standard deviations. Associations between categorical variables were assessed by χ2 test and Fisher exact test. Odds ratios and 95% confidence intervals were calculated using binary logistic regression to separately assess the effect of each independent variable on the odds of AKI. Statistical significance was set at p < 0.05. RESULTS: A total of 74 cases were identified. Out of these 74 cases, 27 had AKI and 47 did not have AKI. Out of the 27 AKI cases, 4 with SRC were excluded. Advanced age, chronic kidney disease, and heart failure were identified as risk factors for AKI development. The predominant cause of AKI was prerenal etiology, accounting for 47.8% (n = 11) of cases. This was followed by cardiorenal syndrome and acute tubular necrosis, accounting for 21.7% and 17.3% of the cases, respectively. Most of the cases with AKI had complete renal recovery 78% (n = 18), whereas 17% (n = 4) had progression of the underlying chronic kidney disease. One patient progressed to end-stage renal disease requiring hemodialysis. CONCLUSIONS: This analysis highlights the risk factors and variable clinicopathological courses of renal involvement in patients with scleroderma. This may range from mild AKI with good prognosis to life-threatening SRC.

Treatment of severe hyponatremia with continuous renal replacement therapy: A case and review of corrective strategies.

Treatment of severely hyponatremic patients with continuous renal replacement therapy (CRRT) presents a unique challenge given the lack of commercial options for hypotonic replacement solutions or dialysate. We report the case of a 55-year-old male who presented with profound, symptomatic hyponatremia in the setting of acute kidney injury (AKI). The patient was found to have a serum sodium concentration of 97 mEq/L because of free water retention that occurred during severe AKI from viral gastroenteritis and rhabdomyolysis. Continuous veno-venous hemofiltration (CVVH) was required for AKI complicated by hyperkalemia, metabolic acidosis, and uremia. To prevent overcorrection of serum sodium, replacement fluids customized to natremic status had to be prepared. Conventional replacement fluid was modified on a daily basis to create hypotonic solutions with successively higher sodium concentrations. Over the course of a week, serum sodium successfully improved in a controlled and safe fashion. This case incorporates and reviews the variety of methods that have been used to safely manage severe hyponatremia with CRRT.

Bilateral adrenal hemorrhage: A rare presentation of catastrophic anti-phospholipid syndrome.

Catastrophic anti-phospholipid syndrome (CAPS) is characterized by microvascular thrombosis in multiple sites leading to multi-organ damage. It is a rare and fatal complication of antiphospholipid syndrome (APS). We present a rare case of CAPS that presented with bilateral (b/l) adrenal hemorrhage making the diagnosis challenging in this otherwise rare disease. A 51-year-old female was initially admitted with abdominal pain and found to have bilateral adrenal hemorrhage. Patient had a fulminant disease course in which she had thrombotic manifestations involving multiple organ systems. This case was especially challenging as the patient's bilateral adrenal hemorrhage was the first manifestation of CAPS; the diagnosis of APS had to be made while treatment for presumed CAPS was emergently commenced for this life-threatening disease. Key to managing this condition is having a high index of suspicion for the diagnosis in patients presenting with multi-organ failure and multiple thromboses and hemorrhage.

Chronic Kidney Disease and Post-Percutaneous Coronary Intervention Mortality in Patients With Left Main and Equivalent Coronary Artery Disease.

BACKGROUND: Patients with chronic kidney disease are underrepresented in registries and in randomized trials of coronary artery disease management. To investigate effects of chronic kidney disease on outcomes of nonemergent percutaneous coronary intervention in patients with left main or left main-equivalent coronary artery disease, we analyzed data from the New York State Percutaneous Coronary Intervention Registry during the calendar year 2015, involving 2,956 elective percutaneous coronary intervention cases. Outcomes of percutaneous coronary intervention in patients with various degrees of chronic kidney disease and stable left main or left main-equivalent coronary artery disease were compared. METHODS: Only patients with left main or left main-equivalent coronary artery disease and elective percutaneous coronary intervention were included in the study cohort. Patients with acute coronary syndromes within 24 hours of the index percutaneous coronary intervention, patients reported to be in shock, and patients with prior coronary artery bypass surgery were excluded from the study cohort. RESULTS: In this cohort, stage 4 or 5 chronic kidney disease, current congestive heart failure, and left main disease remained statistically significant predictors of post-percutaneous coronary intervention mortality. CONCLUSION: Our findings in this large, statewide cohort indicate that advanced kidney disease is associated with markedly increased post-nonemergent percutaneous coronary intervention mortality.

Scleroderma Renal Crisis Associated With Microangiopathic Hemolytic Anemia in a Patient With Seronegative Scleroderma and Monoclonal Gammopathy.

Systemic sclerosis with negative serology, particularly that complicated by scleroderma renal crisis (SRC), is rarely encountered. We describe a patient with seronegative systemic sclerosis who developed acute kidney injury, proteinuria, and hypertensive emergency following motor vehicle-related trauma and in the setting of nonsteroidal anti-inflammatory drug use. Findings on physical examination, imaging, and skin biopsy led to a clinical diagnosis of scleroderma, despite the lack of supportive laboratory data. IgG lambda paraproteinemia was detected on workup. Bone marrow biopsy showed plasmacytosis and trace lambda-restricted plasma cells consistent with monoclonal gammopathy of undetermined significance. Chemotherapy was initially started given concern for myeloma with cast nephropathy but was later stopped after a kidney biopsy revealed thrombotic microangiopathy (TMA). The SRC associated with TMA was ultimately diagnosed, though atypical hemolytic uremic syndrome (aHUS) induced perhaps by monoclonal gammopathy or hypertension was also possible. Captopril and eculizumab were initiated for SRC and aHUS, respectively. Despite therapy, renal function did not recover, and the patient required hemodialysis indefinitely. This case highlights clinical features common to both SRC and aHUS as well as points out a few ways to differentiate between them.

Effect of intensive blood pressure on the progression of non-diabetic chronic kidney disease at varying degrees of proteinuria.

The ideal blood pressure (BP) target for renoprotection is uncertain in patients with non-diabetic chronic kidney disease (CKD), especially considering the influence exerted by pre-existing proteinuria. In this pooled analysis of landmark trials, we coalesced individual data from 5001 such subjects randomized to intensive versus standard BP targets. We employed multivariable regression to evaluate the relationship between follow-up systolic blood pressure (SBP) and diastolic blood pressure (DBP) on CKD progression (defined as glomerular filtration rate decline by 50% or end-stage renal disease), focusing on the potential for effect modification by baseline proteinuria or albuminuria. The median follow-up was 3.2 years. We found that SBP rather than DBP was the primary predictor of renal outcomes. The optimal SBP target was 110-129 mm Hg. We observed a strong interaction between SBP and proteinuria such that lower SBP ranges were significantly linked with progressively lower CKD risk in grade A3 albuminuria or ≥0.5-1 g/day proteinuria (relative to SBP 110-119 mm Hg, the adjusted HR for SBP 120-129 mm Hg, 130-139 mm Hg, and 140-149 mm Hg was 1.5, 2.3, and 3.3, respectively; all p<0.05). In grade A2 microalbuminuria or proteinuria near 0.5 g/day, a non-significant but possible connection was seen between tighter BP and decreased CKD (aforementioned HRs all <2; all p>0.05), while in grade A1 albuminuria or proteinuria <0.2 g/day no significant association was apparent (HRs all <1.5; all p>0.1). We conclude that in non-diabetic CKD, stricter BP targets <130 mm Hg may help limit CKD progression as proteinuria rises.