CEN Case Reports最新文献

An 18-year-old female presented with palpable purpura nine months before her hospital admission, which first appeared 1 month after receiving a COVID-19 vaccine and recurred intermittently. One month prior to admission, she developed macrohematuria, abdominal pain, and a loss of appetite. Occult blood in urine had been noted during high school health check-ups. Upon admission, she continued to have macrohematuria, along with renal dysfunction and a nephritic urinalysis, serum myeloperoxidase-anti-neutrophil cytoplasmic antibodies (MPO-ANCA) positivity. A renal biopsy revealed crescentic glomerulonephritis with mesangial and endocapillary hypercellularity, and dominant IgA deposition and electron-dense deposits in the mesangial regions. The diagnosis was IgA nephropathy (IgAN) or IgA vasculitis with nephritis (IgAVN), with a possible overlap of MPO-ANCA-associated glomerulonephritis. Treatment began with methylprednisolone pulse therapy and prednisolone. After the diagnosis, rituximab (RTX) and avacopan were added to the regimen. Within two months, renal function, hematuria, and MPO-ANCA levels had normalized, and proteinuria was almost fully resolved by 13 months. If IgAN/IgAVN and ANCA-associated vasculitis were indeed triggered by the COVID-19 vaccination in this case, it is plausible that both conditions share similar pathologic mechanisms. This case emphasizes the need for a reliable laboratory method to detect pathogenic ANCA to guide both induction and maintenance therapy. Further investigation into the effectiveness of the ANCA-associated glomerulonephritis treatment protocol including corticosteroids, RTX, and avacopan in managing crescentic IgAN/IgAVN could offer valuable insights into improving patient care.

Anti-glomerular basement membrane (GBM) disease causes rapidly progressive glomerulonephritis (RPGN) and has a high mortality rate from lung hemorrhage or infection as a side effect of immunosuppressive treatment. We report a case in which a patient with anti-GBM disease experienced severe jejunal bleeding due to cytomegalovirus (CMV) enteritis during immunosuppressive treatment. A previously healthy 74-year-old female was admitted to our hospital with severe acute kidney injury due to intrinsic kidney disease. The patient was anuric, and hemodialysis was started. The initial serum anti-GBM antibody level was elevated at 1880 U/mL. Kidney biopsy demonstrated global glomerulosclerosis and diffuse crescent formation on light microscopy. Immunofluorescence revealed focal deposition of IgG along the glomerular capillaries. The patient was diagnosed with RPGN secondary to anti-GBM disease, and oral prednisolone and double filtration plasmapheresis (DFPP) were begun. During treatment, the patient developed recurrent jejunal hemorrhage refractory to endoscopic clipping. Surgical resection of the intestine was performed to control bleeding. There were CMV-positive cells within the resected jejunum on immunohistochemistry. CMV antigens were also detected in the serum. The patient was diagnosed with CMV enteritis and treated with ganciclovir. Ultimately, the patient was discharged home without any neurologic problems on the 285th day of hospitalization. When treating severe anti-GBM antibody disease, there is a dilemma between the effectiveness and the side effects, especially infections, of immunosuppressive therapy. In this case, moderate immunosuppressive therapy with corticosteroids and DFPP without cyclophosphamide contributed to controlling the CMV enteritis and the patient's survival.

A 76-year-old Japanese man was incidentally diagnosed with a pancreatic head tumor on computed tomography after surgery for colon cancer. He underwent pancreatoduodenectomy and was diagnosed with IgG4-related autoimmune pancreatitis. Concurrent chronic kidney disease gradually progressed and chronic hemodialysis was introduced 2 years later. Six months after the introduction of hemodialysis, follow-up abdominal computed tomography revealed marked enlargement of bilateral kidneys compared with previous images. Blood tests revealed persistent high IgG and IgG4 levels, and IgG4-related kidney disease was suspected. Thus, percutaneous kidney biopsy was performed. No evidence of IgG4-related kidney disease was detected, and a diagnosis of diffuse large B-cell lymphoma was made. Six courses of combination chemotherapy consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone was effective, and the patient achieved and maintained complete remission for five years. This case highlights the need to consider the possible development of malignant lymphoma within several years after IgG4-related disease, especially in cases of autoimmune pancreatitis.

Type I and mixed cryoglobulinemic vasculitis differ in pathophysiology, clinical presentation, and therapeutic response. We report a case of refractory cryoglobulinemic vasculitis diagnosed following ischemic non-obstructive coronary artery disease (INOCA). The patient presented with dyspnea, as well as abdominal pain due to ischemic enteritis, purpura, and renal failure requiring dialysis. Despite the patient's IgG λ-type monoclonal gammopathy of undetermined significance (MGUS) and negative hepatitis C virus, the presence of rheumatoid factor (RF) activity and the possibility of IgM involvement were suggested by cryoglobulin analysis and strong glomerular IgM deposition. The condition was diagnosed as mixed cryoglobulinemia, and various immunomodulatory treatments, including methylprednisolone, rituximab and plasmapheresis, were administered without achieving cryoglobulin negativity. However, treatment with bortezomib and dexamethasone ultimately led to cryoglobulin negativity and clinical improvement although the patient was not weaned off dialysis, resulting in remission of the cryoglobulinemic vasculitis. This case suggests that bortezomib, a proteasome inhibitor, may be a promising treatment for refractory cryoglobulinemic vasculitis.

In March 2024, a significant public health issue involving red yeast rice supplements emerged in Japan, which may have been associated with several health problems, including kidney dysfunction. A 74-year-old man without a history of urinary abnormalities developed nephrotic syndrome 10 weeks after starting "Beni-Koji Choleste Help^®," a red yeast rice supplement. Electron microscopy showed subepithelial and intramembranous dense deposits with abnormality of the glomerular basement membrane, which led to the diagnosis of long-standing membranous nephropathy. The diagnosis of phospholipase A2 receptor (PLA2R)-positive primary membranous nephropathy was confirmed via PLA2R-positive immunostaining. Furthermore, there were findings of focal tubular necrosis without unexplained cellular infiltration. Based on previous reports, it was suggested that red yeast rice-related products have triggered the acute tubular necrosis in the patient. His nephrotic syndrome reached partial remission with oral administration of prednisolone at an initial dose of 40 mg/day. This case indicates that membranous nephropathy was coincidentally accompanied by acute tubular necrosis that was potentially associated with red yeast rice-related health foods. It may be reasonable to state that individuals consuming red yeast rice-related health products may develop subclinical focal tubular necrosis even in the absence of overt clinical symptoms or laboratory abnormalities. The currently recognized health issues associated with red yeast rice-related health foods may only represent a small portion of the overall risks.

Several cases of glomerulonephritis occurring after infection with human parvovirus B19 (PVB19) have been reported. However, the pathogenesis and clinicopathological features of PVB19-related glomerulonephritis remain elusive. We describe the case of a 34 year-old woman who showed nephrotic syndrome and microscopic hematuria 10 days after PVB19 infection. Blood pressure and renal function were within normal ranges. Laboratory tests showed positive results for anti-PVB19 immunoglobulin (Ig)M antibody and complement 3 (C3) hypocomplementemia. Antibody to streptolysin O (ASO) was slightly elevated, but bacterial cultures yielded no colonies. Light microscopy of renal biopsy was compatible with membranoproliferative glomerulonephritis (MPGN). Immunofluorescence microscopy showed intense staining for C3 and faint staining for IgG on the glomerular capillary wall and paramesangial area. Electron micrography showed subendothelial electron-dense deposits (EDDs), but hump-shaped subepithelial EDDs were not evident. PBV19-DNA was absent from renal tissue. Moreover, glomeruli showed positive staining for nephritis-associated plasmin receptor (NAPlr) and plasmin activity with similar distribution. Around 6 months after PVB19 infection, levels of anti-PVB19 IgM antibody spontaneously tuned negative with an apparent reduction of proteinuria and improvement of hypocomplementemia, although ASO level remained unchanged. This appears to represent the first description of positive glomerular staining for NAPlr in MPGN after PVB19 infection. Based on a review of 27 cases, including our own case, the MPGN lesions could be attributable to PVB19 infection. Clinicopathological features of this case were incompatible with post-streptococcal acute glomerulonephritis. We presume that a PBV19-derived glomerular pathogen that cross-reacts with anti-NAPlr antibody might be involved in the development of PVB19-related MPGN.

We report the case of a 75-year-old woman who presented with fever, right back pain, paresthesia in the right extremities, erythema, purpura, and nodules. She had previously initiated dialysis due to rapidly progressive glomerulonephritis and was transferred to our hospital. Imaging studies revealed multiple cerebral and splenic infarcts and hemorrhage encapsulating the right kidney, likely due to microaneurysms in multiple renal arteries. High MPO-ANCA titers were observed, and a skin biopsy revealed granulomatous inflammation affecting medium-sized vessels, leading to a diagnosis of granulomatosis with polyangiitis (GPA) and ANCA-associated vasculitis (AAV) involving medium-sized vessels. Treatment began with intravenous pulse steroid therapy (methylprednisolone 1000 mg/day) and subsequent oral prednisolone (PSL) 40 mg (about 0.8 mg/kg) and intravenous cyclophosphamide (IVCY) at 250 mg. While her symptoms improved, she developed severe infections, including candidemia and febrile neutropenia. Consequently, we combined PSL with the C5a receptor antagonist avacopan, which allowed for PSL tapering and stabilized her disease. This case is significant as no previous reports of avacopan's efficacy in AAV with medium-sized vessel involvement suggest its potential effectiveness in such cases.

Granulomatosis with polyangiitis is an ANCA-associated vasculitis that involves small to medium-sized vessels. The extent of renal involvement varies, which is also associated with disease prognosis, with aggressive renal involvement having worse outcomes. Rapidly progressive glomerulonephritis with severe inflammatory features and extensive crescent formation can be challenging to treat. Usually, induction regimes utilize a combination of pulse dose methylprednisolone followed by rituximab or cyclophosphamide. Resistant diseases pose additional treatment challenges, and individualized treatment regimens have been described without accumulated outcome data. Cyclophosphamide, rituximab, azathioprine, methotrexate, and mycophenolate with or without plasmapheresis have been variably used, but there is a lack of consensus on a standardized regime in literature. Our case adds to the existing literature on the treatment-refractory granulomatosis with polyangiitis, which was treated with high-dose corticosteroid in combination with rituximab, low-dose cyclophosphamide, plasmapheresis, and brief use of hemodialysis. It also reiterates that the use of a variety of low-dose cyclophosphamide with rituximab could be beneficial for treatment-refractory cases or patients with severe renal involvement, in addition to better tolerance with low dose cyclophosphamide in comparison with full-dose cyclophosphamide.

Syphilis is an infectious disease caused by the spirochete Treponema pallidum and is increasingly prevalent worldwide, with a rapid increase in reported cases in Japan in recent years. Syphilis is characterized by skin lesions, lymphadenopathy, and hepatosplenomegaly but is rarely manifested by renal disorders, including membranous nephropathy (MN). Neuron-derived neurotrophic factor (NDNF) is an antigen specific for syphilis-associated MN. This report describes a case of NDNF-related MN after syphilis infection in a 42-year-old male who developed nephrotic syndrome 3 months after infection. Renal biopsy under light microscopy revealed mild mesangial proliferation without spike formation in the glomerular basement membrane. Immunofluorescence and electron microscopy revealed granular deposits of IgM, IgG, C1q, and C3 on the capillary walls, with subepithelial hump-like electron-dense deposits (EDDs), consistent with stage I MN. Immunohistochemistry confirmed the presence of NDNF. In contrast, other common antigens related to primary MN, such as M-type phospholipase A2 receptor and thrombospondin type I domain-containing 7A, were negative on mass spectrometry. The patient achieved remission with antibiotic therapy alone. This case and the literature review on NDNF-related MN highlight the relevance of NDNF as a syphilis-associated MN antigen and demonstrate that antibiotic therapy alone without immunosuppressive drugs can lead to remission. Mass spectrometry can accurately identify MN antigens; however, immunostaining is more effective in cases where EDDs are segmental and antigen concentration is low. Our findings indicate that NDNF testing should be performed in cases of proteinuria associated with syphilis to help guide antibiotic therapy and reduce immunosuppression.