Case Reports in Nephrology最新文献

Concomitant with nephrotic syndrome and multicentric castleman's disease (MCD) has only been described in a limited number of small studies and case reports. Among those, none confirmed the renal pathology prior to the onset of MCD, and none of the cases had a history of nephrotic syndrome. A 76 year-old Japanese man visited a nephrologist because of incident nephrotic syndrome. He had previously experienced three episodes of nephrotic syndrome, the last one 13 years ago, and had been diagnosed with membranous nephropathy by renal biopsy. Apart from these previous episodes, he also suffered from systemic lymphadenopathy, anemia, elevated C-reactive protein, polyclonal hypergammopathy, and elevated interleukin (IL)-6. An inguinal lymph node biopsy revealed CD138-positive plasma cells in the interfollicular region. Based on these findings, MCD was diagnosed. Renal biopsy indicated primary membranous nephropathy with spike lesions and bubbling in the basement membranes and deposition of immunoglobulin (Ig) G, IgA, IgM, and phospholipase A2 receptor along the glomerular basement membrane. Corticosteroid monotherapy successfully reduced the edema, proteinuria, and IL-6, but hypoalbuminemia was not sufficiently improved due to castleman's disease and remission of the nephrotic syndrome was not achieved. Later, tocilizumab was administered for remission induction in another facility. To the best of our knowledge, this represents the first report of Castleman's disease with previously diagnosed membranous nephropathy. This case does not provide a causal mechanism for the pathophysiology, but it may be worth suggesting possible involvement of MCD as a trigger for recurrence of membranous nephropathy.

Antitubular basement membrane (anti-TBM) antibody disease is an extremely rare disorder. It may be idiopathic or secondary to exposure of the proximal tubular basement membrane, triggered by tubular injury due to acute pyelonephritis, acute allergic interstitial nephritis, or kidney allograft rejection. The histopathology of anti-TBM antibody disease is characterized by strong linear deposits of IgG with complement C3 along the proximal tubular cell basement membranes. The staining is restricted to proximal tubules. Currently, a kidney biopsy with these pathognomonic findings is the only diagnostic method. Serological testing and titers for anti-TBM antibodies are not clinically standardized. Our patient had pyelonephritis and possibly acute allergic interstitial nephritis as a result of nivolumab infusion. The kidney biopsy demonstrated dense interstitial infiltrates of neutrophil-rich interstitial inflammation, neutrophilic casts, and neutrophilic tubulitis consistent with acute pyelonephritis, as well as areas of mixed inflammation with lymphocytic tubulitis suggesting concurrent acute interstitial nephritis. The presence of linear IgG staining along proximal but not distal tubular basement membranes was diagnostic of anti-TBM antibody disease, favored to be due to both triggers. The patient was treated with discontinuation of nivolumab, intravenous antibiotics, and corticosteroids and was supported with hemodialysis. After 6 weeks, the patient's kidney function recovered enough to discontinue hemodialysis and had significant renal improvement.

We experienced a case of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis after Moderna COVID-19 vaccination. An 82-year-old woman developed pyrexia and general malaise one month after her third booster vaccine, and the symptoms persisted. Blood testing revealed inflammation, a high level of MPO-ANCA, and microscopic hematuria. MPO-ANCA-associated vasculitis was diagnosed by renal biopsy. The symptoms improved with steroid therapy. Common adverse reactions to mRNA vaccines against COVID-19 include pyrexia and general malaise, but MPO-ANCA-associated vasculitis can also occur. If pyrexia, prolonged general malaise, urinary occult blood, or renal impairment is observed, the onset of MPO-ANCA-associated vasculitis should be considered.

PD-1/PD-L1 inhibitors such as pembrolizumab have radically improved the prognosis for many patients with advanced malignancies. Although revolutionary, its use can be complicated and limited by various immune-related adverse effects. Effective management depends on early recognition and prompt intervention. Herein, we describe a unique syndrome of hypercalcemia, with associated acute renal injury and hypoxic respiratory failure that was responsive to corticosteroids suggestive of immunotoxicity from pembrolizumab.

Ogilvie's syndrome, also known as acute colonic pseudo-obstruction (ACPO), is a rare, nonobstructive dilation of the colon of unclear etiology. We present the case of a patient who presented with Ogilvie's syndrome and significant hypokalemia due to colonic loss despite repletion. This case report demonstrates the difficulty in diagnosis, treatment, and outcome.

Mycobacterium tuberculosis (MTB) is an under-recognised cause of genitourinary disease. IgA nephropathy (IgAN), a leading cause of glomerulonephritis worldwide, has been described as a rare consequence of disseminated MTB infection. In this case report, we present the first case of MTB associated IgAN in Africa. Finding IgAN on kidney biopsy in an MTB endemic area should prompt a thorough investigation for MTB to increase the chance of remission of IgAN and prevent inappropriate use of immunosuppression.

We present the case of an 86-year-old Caucasian male with an 11-year history of low-grade chronic lymphocytic leukemia (CLL) presenting with nephrotic syndrome (NS). Renal biopsy findings showed a diffuse mesangial and endocapillary proliferative glomerulonephritis (GN) lesion with fine granular deposits, consistent with a rare morphologic variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID)-lambda light chain (LC) only. Monthly combination therapy of rituximab (500 mg/m² on day 1), fludarabine (30 mg/m² on days 1-3), and cyclophosphamide (750 mg/m² on days 1-3) was administered. Five courses of this regimen resulted in hematological remission, as well as a partial renal response with a reduction in the spot urine protein-to-creatinine ratio (UPCR) of 815.3 mg/g (reduction > 50% proteinuria without improvement in kidney function). This condition is a rare morphological variant of PGNMID, poorly described in CLL patients. We review the literature and suggest that this case provides sheds light on the unknown pathophysiological mechanisms of monoclonal immunoglobulins (MIg)-mediated glomerular damage in CLL patients, and may be helpful for the investigation of a more effective treatment.

Central diabetes insipidus (DI) is characterized by a deficiency in arginine vasopressin (AVP), an antidiuretic hormone leading to excessive free water loss in the urine and hypernatremia. Central DI can be the first presentation of several occult diseases. However, patients with central DI who have functioning thirst mechanisms and access to water may initially exhibit normal sodium levels. We report a 57-year-old woman who was admitted to the hospital due to cholangitis. Her initial serum sodium was normal and she rapidly developed severe hypernatremia after fluid restriction. The results of the laboratory workup indicated DI, which dramatically responded to desmopressin. MRI showed an ill-defined faint hyper signal intensity in T1, T2/FLAIR lesions involving the bilateral hypothalamus. The histopathological findings confirmed the diagnosis of Langerhans cell histiocytosis (LCH) with multiorgan involvement. Serum sodium returned to normal after receiving desmopressin and water replacement therapy.

Malignant pleural mesothelioma is rarely associated with nephrotic syndrome. Cryoglobulinemia is found in various pathological statuses, such as hepatitis C virus infection but rarely in malignant neoplasms. We recently encountered a patient with malignant mesothelioma coincident with nephrotic syndrome and cryoglobulinemia in the course of chemotherapy. A 60-year-old man employed as a building painter was diagnosed with malignant mesothelioma by lung biopsy two years earlier and was started on chemotherapy. Nivolumab seemed effective in controlling mesothelioma, but skin immune-related adverse events occurred during the course of treatment. After discontinuation of nivolumab and administration of gemcitabine as an alternative therapy, the patient was referred to a nephrologist because of the subsequent development of edema, renal injury, and proteinuria. Following the investigation, he was diagnosed with nephrotic syndrome and cryoglobulinemia with C4-dominant cold activation. However, a percutaneous renal biopsy could not be performed due to persistent severe cough induced by pleural involvement. The patient died a little over three years after the pathological diagnosis of pleural mesothelioma. Our case had three key features nephrotic syndrome was possibly associated with malignant mesothelioma; cryoglobulinemia occurred in malignant mesothelioma; and concomitant nephrotic syndrome and cryoglobulinemia occurred after chemotherapy. Unfortunately, our rare case lacks a basis in renal pathology or evidence of links between the pathogenesis of malignant mesothelioma, cryoglobulinemia, and nephrotic syndrome. This case does not provide a causal mechanism, but may be worth adding to the case list as one of the rare renal involvement in a patient with malignant mesothelioma.