A case of immunoglobulin G4-related disease complicated by atopic dermatitis responsive to upadacitinib treatment.

Immunoglobulin G4-related disease (IgG4-RD) is mainly treated with glucocorticoids. In many cases, this disease is resistant to glucocorticoids, and their toxicity can be a problem. We encountered a patient with IgG4-RD affecting multiple organs (such as the skin, lung, and lacrimal gland), who had comorbidities, including atopic dermatitis and diabetes.

A case of Buerger's disease with vasculopathy and skin fibrosis requiring differential diagnosis from systemic sclerosis.

Buerger's disease is characterised by peripheral ischaemia due to occlusion of small- and medium-sized arteries in the extremities. This report describes a case of Buerger's disease in a 51-year-old male who presented with findings resembling systemic sclerosis (SSc). The patient exhibited Raynaud's phenomenon in year 2015, which developed to skin hardening, nail avulsion, and ulceration of the right fingers in year 2018.

Modifying T cell phenotypes using TYK2 inhibitor and its implications for the treatment of systemic lupus erythematosus.

Objectives: The tuning effects of JAK/TYK2 inhibitors on the imbalance between T follicular helper (Tfh) and T regulatory (Treg) cells, related to systemic lupus erythematosus (SLE) pathogenesis, were investigated using human peripheral blood samples.

Methods: Peripheral blood mononuclear cells from untreated patients with SLE and healthy controls were analysed. Tfh1 cells were identified in nephritis tissue, and the effect of Tfh1 cells on B-cell differentiation was examined by coculturing naïve B cells with Tfh1 cells.

Sarcomatoid malignant pleural mesothelioma: a case of long-term recurrence-free survival following curative intent surgery alone.

Background: Curative intent surgery may be indicated for some patients with resectable early stage malignant pleural mesothelioma (MPM). However, sarcomatoid MPM is a highly aggressive subtype for which curative intent surgery is generally not recommended.

Case Presentation: We present the case of a 63-year-old man who presented with dyspnea and chest tightness.

Outcomes and pathologic response of primary lung cancer treated with tyrosine kinase inhibitor/immune checkpoint inhibitor before salvage surgery.

Purpose: Advances in primary lung cancer drug therapy have extended patients' survival, including patients with stage IV disease. This study assessed the safety and effectiveness of salvage surgery following tyrosine kinase inhibitor (TKI) or immune checkpoint inhibitor (ICI) therapy in primary lung cancer.

Methods: A retrospective chart review was conducted of 2050 primary lung cancer surgeries performed at our institution between 2012 and 2022.

Peritumoral Fat Content Identified Using Iterative Decomposition of Water and Fat with Echo Asymmetry and Least-squares Estimation (IDEAL) Correlates with Breast Cancer Prognosis.

Purpose: Adipocytes around aggressive breast cancer (BC) are less lipid different from naive adipocytes (cancer-associated adipocytes, CAAs), and peritumoral edema caused by the release of cytokines from CAAs can conduce to decrease the peritumoral fat proportion. The purpose of this study was to correlate peritumoral fat content identified by using iterative decomposition of water and fat with echo asymmetry and least-squares estimation (IDEAL) with lymph node metastasis (LNM) and recurrence-free survival (RFS) in BC patients and to compare with T2-weighted (T2WI) and diffusion-weighted images (DWI) analyses.

Methods: This retrospective study consisted of 85 patients who were diagnosed with invasive carcinoma of breast and underwent breast MRI, including IDEAL before surgery.

A delayed diagnosis of fascioliasis: The importance of appropriate fecal diagnostic method.

Fascioliasis, a zoonotic helminthiasis, occurs sporadically in Japan. In this report, we describe a case of fascioliasis that was initially difficult to diagnose because the fecal examination method was negative for the Fasciola sp. eggs.

A Pediatric Case of Disseminated Bartonella henselae Infection Accompanied by Multiple Intracranial Lesions.

We report a pediatric case of disseminated Bartonella henselae infection accompanied by multiple intracranial lesions. The patient developed multiple intracranial lesions despite treatment with azithromycin and gentamicin. After switching to rifampicin, the clinical symptoms of the patient improved.

Differential expression of IFN-α, IL-12 and BAFF on renal immune cells and its relevance to disease activity and treatment responsiveness in patients with proliferative lupus nephritis.

Objective: Since molecularly targeted therapies are emerging for treating lupus nephritis (LN), this study aimed to assess the immunohistochemical findings of the cytokines in renal tissue and their pathological and clinical relevance in LN.

Methods: Fifty patients with proliferative LN formed the case group; 5 with LN class II, IgA nephropathy and 10 with idiopathic haematuria were enrolled as controls. Immunohistochemical analysis for CD3, CD20, interferon (IFN)-α, interleukin (IL)-12/p40 and B-cell activating factor (BAFF) was performed by scoring the number of positive cells/area of the cortex.

CD34-Positive Spindle Cell Tumor With Mutation: An Unusual Spindle Cell Variant of Sinonasal Glomangiopericytoma.

Sinonasal glomangiopericytoma is an uncommon mesenchymal tumor with a perivascular myoid phenotype, which is categorized as a borderline/low-grade malignant soft tissue tumor by the current World Health Organization Classification of Head and Neck tumors. Here, we present the case of a 53-year-old woman with an unusual spindle cell morphology of sinonasal glomangiopericytoma arising in the nasal cavity, mimicking solitary fibrous tumor. Microscopically, the tumor showed a cellular proliferation of spindle cells in fascicles including a focal long sweeping arrangement or whorls, or with a storiform growth pattern, associated with hemangiopericytoma-like gaping blood vessels embedded in a fibrous stroma.

Immunoglobulin G deposition on proximal tubules and the tubular basement membrane in acute tubular injury complicated with focal segmental glomerulosclerosis (FSGS): A possible prediction tool for subclinical FSGS.

Immunofluorescent deposition of immunoglobulin G (IgG) in the tubular basement membrane (TBM) has been evaluated in the diagnosis of various diseases; however, few studies have investigated the immunofluorescence of acute tubular injury (ATI). Herein, we attempted to clarify IgG expression in the proximal tubular epithelium and TBM in ATI due to various causes. Patients with ATI with nephrotic-range proteinuria, including focal segmental glomerulosclerosis (FSGS, n = 18) and minimal change nephrotic syndrome (MCNS, n = 8), ATI with ischemia (n = 6), and drug-induced ATI (n = 7), were enrolled.

TGF-β3 in differentiation and function of Tph-like cells and its relevance to disease activity in patients with systemic lupus erythematosus.

Objectives: T peripheral helper (Tph) cells have major roles in pathological processes in SLE. We sought to clarify the mechanisms of Tph cell differentiation and their relevance to clinical features in patients with SLE.

Method: Phenotypes and functions of Tph cell-related markers in human CD4+ T cells purified from volunteers or patients were analysed using flow cytometry and quantitative PCR.

A case of giant cell arteritis complicated by polymyalgia rheumatica with scalp necrosis and refractory jaw claudication.

Scalp necrosis is a rare complication of giant cell arteritis (GCA); however, it is a predictor of severe disease. In this case study, a patient presented with GCA complicated by polymyalgia rheumatica with scalp necrosis. An 86-year-old woman was admitted to the hospital for pulsating headache, scalp pain, jaw claudication, and generalised pain.

Remission of Granulomatosis with Polyangiitis Only After Resection of a Pulmonary Nodule.

Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous lesions and is classified as ANCA-associated vasculitis (AAV). We herein report a case of GPA that was remitted by resection of a pulmonary lesion without immunosuppressive therapy. We detected activated neutrophils and neutrophil extracellular traps (NET) formation in resected lung tissue by immunofluorescence.

Catastrophic antiphospholipid syndrome with Epstein-Barr virus-associated hemophagocytosis: A clinicopathological conference.

Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of antiphospholipid syndrome associated with multiorgan thrombosis in a short term. We present the case of a 14-year-old immunocompetent girl who developed renal, intestinal, and pulmonary infarction; thrombocytopenia; and hemolytic anemia within 1 week. She was diagnosed with thrombotic microangiopathy.

Salvage Pleurectomy/Decortication After Immunotherapy for Sarcomatoid Malignant Pleural Mesothelioma.

Sarcomatoid malignant pleural mesothelioma (MPM) is a highly aggressive malignant tumor. Surgery may not be recommended, and chemotherapy is less effective. More recently, immunotherapy has become a new standard treatment of care for advanced MPM across all histologic subtypes.

Eosinophilic granulomatosis with polyangiitis exhibits T cell activation and IgG4 immune response in the tissue; comparison with IgG4-related disease.

Objective: To study the pathophysiological differences of EGPA and IgG-related disease (RD) by clarifying their clinical, pathological and immunological features.

Methods: Clinical and pathological findings were compared in patients with EGPA and IgG-RD. Peripheral blood mononuclear cells were used for comprehensive flow cytometric analysis.

A case of simultaneous onset of highly active systemic lupus erythematosus and IgG4-related renal disease.

The patient was a 73-year-old woman who had hair loss, purpura, and numbness of the soles for past 1 year. Three months prior, she was diagnosed with interstitial lung disease (ILD) and was admitted to our department. She was diagnosed with systemic lupus erythematosus (SLE) based on positive antinuclear antibodies 1280× (speckled type), hair loss, low white blood cell count, positive anti-cardiolipin and anti-ds-DNA antibodies, and lupus retinopathy.

Rituximab in remission induction and maintenance therapy for microscopic polyangiitis associated with gastric cancer: A case report.

We herein report a case of a patient with gastric cancer-associated microscopic polyangiitis (MPA) who was treated with combination glucocorticoids and rituximab (RTX) for remission induction and maintenance, and finally to discontinue glucocorticoids without recurrence of gastric cancer or MPA in a year. A 69-year-old man was suspected of having MPA because of fever, high C-reactive protein levels, neuritis, and a high titer of myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA). Upper gastrointestinal endoscopy indicated early-stage gastric cancer, for which he underwent surgery preceded by immunosuppressive therapy for vasculitis.

Focal segmental glomerulosclerosis and concurrent glomerular microangiopathy after long-term imatinib administration.

A 79-year-old Japanese man was admitted to our hospital because of proteinuria and kidney dysfunction. He was diagnosed with chronic myeloid leukemia 13 years before and was treated with imatinib. Deep molecular response was achieved but he developed 1+ proteinuria in the first year, which gradually worsened thereafter.

Nephrotic syndrome with acute kidney injury due to focal segmental glomerulosclerosis following long-term treatment with minodronate.

Although bisphosphonates are well known to cause kidney disease, there are very few published cases of focal segmental glomerulosclerosis (FSGS) following treatment with minodronate. Here we report the case of an 86-year-old woman who developed acute kidney injury and nephrotic syndrome after receiving monthly oral minodronate for 24 months. Kidney biopsy revealed cellular variant FSGS.