Progression to End-Stage Renal Disease Due to IgG4-Related Nephritis Refractory to Rituximab.

An 81-year-old woman was referred to nephrology for a follow-up on progressive chronic kidney disease. She has a past medical history of hypertension, T2DM, breast cancer, and secondary hyperparathyroidism related to renal disease. A renal biopsy showed patchy interstitial fibrosis and tubular atrophy with an increased number of IgG4-positive plasma cells. A diagnosis of IgG4-related kidney disease was made based on clinical presentation and pathology. The patient ultimately required the initiation of hemodialysis, despite the administration of steroids and rituximab.

Triple hit to the kidney-dual pathological crescentic glomerulonephritis and diffuse proliferative immune complex-mediated glomerulonephritis: A case report.

BACKGROUND: Anti-glomerular basement membrane (GBM) disease is a rare rapidly progressive glomerulonephritis, frequently associated with alveolar hemorrhage in the lungs and involving the kidney by crescentic glomerulonephritis. It has been described in association with other glomerulonephritides [such as anti-neutrophilic antibody (ANCA)-glomerulonephritis, membranous nephropathy, and immunoglobulin (Ig)A nephropathy]. CASE SUMMARY: Herein we present an unusual case of concurrent anti-GBM disease, ANCA-associated crescentic glomerulonephritis and diffuse proliferative immune complex mediated glomerulonephritis with predominant staining for IgA and C3 by immunofluorescence. The patient is a 46-year-old Caucasian male who presented to the emergency department with acute onset of flank pain and was found to have high serum creatinine levels of 15 mg/dL, proteinuria, and hematuria. He rapidly deteriorated and became anuric. He was found to have high anti-GBM antibodies titers (151 units) and high anti-neutrophil cytoplasmic-ANCA. Despite prompt and early treatment, the patient's condition worsened, and he succumbed to his illness. CONCLUSION: Our case emphasizes the importance of a renal biopsy in anti-GBM disease, even in the presence of positive serum anti-GBM antibodies, to identify other potential causes of rapidly progressive glomerulonephritis. The challenge in treating such cases lies in the different therapy modalities.

Two Case Reports of Patients With Transverse Myelitis as a Complication of SARS-CoV-2 Infection.

Transverse myelitis is a nontraumatic spinal cord injury that presents with sudden onset weakness, sensory deficits, and autonomic dysfunction. It can be caused by multiple etiologies including malignancy, autoimmune disorders, viral, bacterial, or fungal infections, and environmental factors. In this article, we describe cases of two elderly male patients affected by the SARS-CoV-2 virus. Patients did not exhibit classic or had only mild classic symptoms of SARS-CoV-2 infection; however, both patients developed transverse myelitis. Patients were treated with intravenous steroids and therapeutic plasmapheresis, achieving partial improvement. The study aimed to understand rare complications like transverse myelitis of SARS-CoV-2 infection and treatment accordingly.

Medical Management of Advanced Oxalate Nephropathy Secondary to Gastric Bypass Surgery.

A 73-year-old Caucasian female with a history of obesity status post Roux-en-Y gastric bypass (RYGB) surgery presented with generalized weakness and was found to have acute kidney injury (AKI) with a creatinine peak of 9.1 mg/dL above her baseline of 1.2 mg/dL, and anemia with hemoglobin 5.7 g/dl. Kidney biopsy revealed oxalate nephropathy likely related to gastric bypass surgery four years prior. RYGB is a strong risk factor for hyperoxaluria, nephrolithiasis, and oxalate nephropathy which often progresses to end-stage renal disease (ESRD). Meaningful treatment strategies for this disease entity are lacking. We present a case in which dietary and pharmacological management without the use of renal replacement therapy resulted in stabilization of chronic kidney disease (CKD) stage 5 for seven years at the time of this writing.

Treatment of fibrillary glomerulonephritis with use of repository corticotropin injections.

Fibrillary glomerulonephritis (FGN) is a rare idiopathic condition linked to malignancy, autoimmune disorders, monoclonal gammopathies and hepatitis C virus. It usually has a poor prognosis, resulting in progression to end-stage renal disease within a few years, given the lack of standardized treatment. Repository corticotrophin (RC) injections are approved for use in a variety of nephrotic syndromes, but are not routinely considered for treatment of FGN. We present a case in which a patient with FGN began treatment with RC 3 months after diagnosis. The patient has attained partial remission with complete resolution of nephrotic syndrome and stabilization of renal function.

Cardiac Sarcoidosis: A Review of Contemporary Challenges in Diagnosis and Treatment.

Sarcoidosis is a systemic disease characterized by noncaseating granulomas and is often a diagnosis of exclusion. The actual prevalence of cardiac sarcoidosis (CS) is unknown, as studies have demonstrated mixed data. CS may be asymptomatic and is likely more frequently encountered than previously thought. Sudden death may often be the presenting feature of CS. Most deaths attributed to CS are caused by arrhythmias or conduction system disease, and congestive heart failure may occur. Current expert consensus on diagnosis of CS continues to rely on endomyocardial biopsy, in the absence of which, histologic proof of extracardiac sarcoid involvement is necessitated. Emergence of newer noninvasive imaging modalities such as cardiac magnetic resonance imaging and positron emission tomography, have become increasingly popular tools utilized in patients with both clinical and asymptomatic CS, and have demonstrated good diagnostic capability. The main therapeutic approaches in patients with CS can be broadly divided into the following 2 categories: pharmacological management and invasive or device oriented. However, much remains unknown about the optimal screening protocols of asymptomatic patients with extracardiac sarcoidosis and treatment of biopsy-proven CS. Our knowledge about CS has amplified significantly over the last 30 years and the growing realization that this process is often asymptomatic is paving the way for better screening protocols and earlier detection of this serious condition.

Oxymorphone Hydrochloride Extended-Release (OPANA®) Associated With Acute Kidney Injury in a Chronic Pain Patient: A Case Report.

Oxymorphone hydrochloride extended-release (OPANA®) is an opioid prescribed for the treatment of moderate-to-severe chronic pain. Kidney injury related to its use has not previously been reported. We present a case of a chronic pain patient with underlying chronic renal insufficiency who developed superimposed acute kidney injury when his opioid analgesic was changed from morphine sulfate extended-release to OPANA. Electron microscopy of his renal tissue revealed lamellated podocytes typically seen with drug-induced phospholipidosis.

Necrotizing crescentic glomerulonephritis related to sarcoidosis: a case report.

INTRODUCTION: Renal injury due to sarcoidosis develops in less than a quarter of patients with this systemic disease. In most cases, granulomatous tissue alters the production of vitamin D, which leads to hypercalciuria, nephrocalcinosis, and nephrolithiasis. Granulomatous interstitial nephritis is another well-recognized pathological process associated with sarcoidosis. However, a glomerular pathology is very rarely noted, and only a few cases are reported to have cellular crescentic glomerulonephritis. CASE PRESENTATION: We describe the case of a 26-year-old African American woman with systemic sarcoidosis, with a unique constellation of renal lesions, including noncaseating epithelioid granulomatous necrotizing interstitial nephritis, cellular crescent formation, and necrotizing vasculitis. Immunosuppressive therapy was helpful for alleviating her nephrotic syndrome and maintaining the stability of her renal function over a 30-month period. CONCLUSION: Glomerular involvement of sarcoidosis needs to be considered in the differential diagnosis in cases of rapidly progressive glomerular nephritis.

Calciphylaxis in Patients With Preserved Kidney Function.

Calcific uremic arteriolopathy (CUA), also known as calciphylaxis, is a devastating disease typically seen in patients with end stage renal disease. It manifests as extremely painful symmetrical wounds resistant to surgical and medical interventions. The prevalence of CUA among hemodialysis dependent patients was found to be as high as 4.1%. The management of patients with CUA requires a multidisciplinary approach by the medical team, yet often results in a low rate of successful outcomes. Recently, non-uremic calciphylaxis (NUC) has been described in the absence of kidney disease. Limited knowledge exists on the management of NUC and the outcomes of this condition. Herein we describe three clinical scenarios of patients diagnosed with NUC in the absence of permanent or prolonged acute renal pathology. The reporting of successful and fruitless therapeutic interventions for wound management in NUC is important for compiling the evidence of effective therapeutic strategies.

Lupus nephritis and end-stage kidney disease.

Systemic lupus erythematosus (SLE) is a multisystem disease affecting many organs. Varying degrees of renal involvement are seen in up to 60% of adults with SLE, and severe lupus nephritis (LN) (World Health Organization class III and above) progresses to end-stage kidney disease (ESKD) within 15 years of diagnosis in 10% to 30% of patients. In fact, renal injury is the most important predictor of mortality in patients with SLE. Identifying patients at risk of progression to ESKD and providing them with aggressive and appropriate immunosuppressive therapy are important factors that affect the morbidity and mortality of LN patients. Management of LN-related ESKD requires attention to persistent activity of SLE and need for continuous immunosuppressive treatment because a decrease in SLE activity in this population can improve their outcome.

Wound Healing in Patients With Impaired Kidney Function.

Renal impairment has long been known to affect wound healing. However, information on differences in the spectrum of wound healing depending on the type of renal insufficiency is limited. Acute kidney injury (AKI) may be observed with different wound types. On one hand, it follows acute traumatic conditions such as crush injury, burns, and post-surgical wounds, and on the other hand, it arises as simultaneous targeting of skin and kidneys by autoimmune-mediated vasculitis. Chronic kidney disease (CKD) and end-stage renal disease (ESRD) often occur in older people, who have limited physical mobility and predisposition for developing pressure-related wounds. The common risk factors for poor wound healing, generally observed in patients with CKD and ESRD, include poorly controlled diabetes mellitus, neuropathy, peripheral vascular disease, chronic venous insufficiency, and aging. ESRD patients have a unique spectrum of wounds related to impaired calcium-phosphorus metabolism, including calciphylaxis, in addition to having the risk factors presented by CKD patients. Overall, there is a wide range of uremic toxins: they may affect local mechanisms of wound healing and also adversely affect the functioning of multiple systems. In the present literature review, we discuss the association between different types of renal impairments and their effects on wound healing and examine this association from different aspects related to the management of wounds in renal impairment patients.

Nonobstructive hydronephrosis due to social polydipsia: a case report.

INTRODUCTION: Excessive fluid intake can lead to water intoxication, electrolyte abnormalities, exacerbation of heart failure and anatomical changes in the urinary tract that may present diagnostic and therapeutic challenges for patients and physicians. Although the development of nonobstructive hydronephrosis is recognized in patients with central and nephrogenic diabetes insipidus, pregnancy or psychiatric polydipsia, it is rarely a diagnostic consideration in healthy individuals with excessive fluid ingestion. We now present what we believe to be the first report of nonobstructive hydronephrosis associated with social polydipsia. CASE PRESENTATION: A 53-year-old African-American woman with moderate back pain was found to have bilateral moderate hydronephrosis and hydroureter by abdominal computed tomography. She underwent ureteral stent placement followed by exploratory laparoscopy with lysis of adhesions and a right oophorectomy, without resolution of the nonobstructive hydronephrosis. A careful assessment revealed a social habit of consuming approximately 5.5L of fluid daily in an effort to remain hydrated in accordance with public health service announcements. It was recommended that the patient reduce her fluid intake. A repeat ultrasound after six weeks revealed complete resolution of the bilateral hydronephrosis and hydroureter. CONCLUSION: Recognition of the nonobstructive nature of hydronephrosis caused by polydipsia in healthy individuals is important to prevent unnecessary interventions.

Lowering serum uric acid to prevent acute kidney injury.

Epidemiological, experimental and clinical studies support a role for uric acid in acute kidney injury (AKI). We discuss how the conventional role of uric acid in AKI has now evolved from intratubular crystal deposition to pro-inflammatory, anti-angiogenic and immunological function. Data from recent studies are presented to support the hypothesis that uric acid may have a role in AKI via a crystal-independent process in addition to its traditionally accepted role to induce injury via crystal-dependent pathways.

Autoimmune encephalopathy associated with thyroid autoantibodies as the cause of reversible cognitive impairment.

We herewith describe a patient with acute confusion, expressive aphasia and generalized seizures. A through workup excluded most causes of encephalopathy. He was, however, found to have TSH=18.6 MIU/ml, T3reverse=0.44nmol/L, T4=0.8ng/dl and Anti-Thyroid-Peroxidase AB titer >1000 IU/ml. Based on the above findings the patient was diagnosed with Hashimoto's encephalopathy and his mental status showed dramatic improvement (MMS 30/30) with high dose prednisone. Hashimoto's encephalopathy is rare disorder of presumed autoimmune origin characterized by cognitive decline, seizures, neuro-psychiatric symptoms, high titers of Anti-Thyroid-Peroxidase AB, and a positive response to steroids.

Takotsubo cardiomyopathy as a potential cause of long QT syndrome and torsades de pointes.

Takotsubo cardiomyopathy is an increasingly reported syndrome characterized by transient apical left ventricular dysfunction in absence of flow limiting coronary artery disease. Prolongation of QT interval associated with Takotsubo cardiomyopathy has been previously reported in published case series but none of these series reported occurrence of torsades de pointes. Reported here is a case of Takotsubo cardiomyopathy resulting in QT prolongation and torsades de pointes in an emotionally distressed woman. The patient had a complete recovery of cardiac function in a week. Takotsubo cardiomyopathy should be considered among the causes of long QT syndrome and torsades de pointes.