A case of antiepiligrin cicatricial pemphigoid successfully treated by plasmapheresis

We report the case of a 73-year-old Japanese woman suffering from antiepiligrin (laminin 5) cicatricial pemphigoid (CP) with typical clinical and immunopathological features. Histologically, the lesional mucous membrane showed a subepidermal blister formation. When indirect immunofluorescence techniques with skin split by 1 M NaCl as the substrate were used, the patient's serum reacted only to the dermal side. Immunoprecipitation studies demonstrated that the patient's serum contained IgG autoantibodies directed against a set of polypeptides that corresponded to epiligrin (laminin 5). After corticosteroids and immunosuppressive agents had been administered systemically, the patient's autoantibody titer decreased and the cutaneous and mucosal blister formations were suppressed. However, the ocular lesions persisted in spite of these therapeutic regimens. After combining these treatments with double-filtration plasmapheresis, the ocular lesions improved and showed almost no progression. Plasmapheresis may thus present a new option for the treatment of CP.     

Stability of masticatory movements after placement of implant-supported denture

Odontology - The purpose of this study was to clarify the stability of masticatory movement after placement of implant-supported denture. Fourteen patients (patient group) with mandibular implants...     

Increased synthesis of anti-tuberculous glycolipid immunoglobulin G (IgG) and IgA with cavity formation in patients with pulmonary tuberculosis

Tuberculous glycolipid (TBGL) antigen is a cell wall component of Mycobacterium tuberculosis and has been used for the serodiagnosis of tuberculosis. We investigated correlations between the levels of anti-TBGL antibodies and a variety of laboratory markers that are potentially influenced by tuberculous infection. Comparisons between patients with cavitary lesions and those without cavitary lesions were also made in order to determine the mechanism underlying the immune response to TBGL. Blood samples were obtained from 91 patients with both clinically and microbiologically confirmed active pulmonary tuberculosis (60 male and 31 female; mean age, 59 ± 22 years old). Fifty-nine patients had cavitary lesions on chest X-rays. Positive correlations were found between anti-TBGL immunoglobulin G (IgG) and C-reactive protein (CRP) (r = 0.361; P < 0.001), between anti-TBGL IgA and soluble CD40 ligand (sCD40L) (r = 0.404; P < 0.005), between anti-TBGL IgG and anti-TBGL IgA (r = 0.551; P < 0.0000005), and between anti-TBGL IgM and serum IgM (r = 0.603; P < 0.00000005). The patients with cavitary lesions showed significantly higher levels of anti-TBGL IgG (P < 0.005), anti-TBGL IgA (P < 0.05), white blood cells (P < 0.01), neutrophils (P < 0.005), basophils (P < 0.0005), natural killer cells (P < 0.05), CRP (P < 0.0005), KL-6 (sialylated carbohydrate antigen KL-6) (P < 0.0005), IgA (P < 0.05), and sCD40L (P < 0.01). The observed positive correlations between the anti-TBGL antibody levels and inflammatory markers indicate the involvement of inflammatory cytokines and NKT cells in the immunopathogenesis of pulmonary tuberculosis.     

Multimodal treatment for adrenal metastases from hepatocellular carcinoma (HCC)

The patients with hepatocellular carcinoma (HCC) with adrenal metastases are often accompanied with the metastasis from other sites, and their prognosis is poor. After 1999, we examined the prognosis and efficacy of the seven patients with drenal metastases from HCC. Four patients were surgically treated, and three of them received radiation therapy (RT). All of the 7 patients were men and the mean age was 72 years old (range: 53-77 years old). The mean interval from the initial treatment of hepatocellular carcinoma to the adrenal metastases was 46 months (1-95 months). If there was a good control observed in the intrahepatic lesion with no metastases besides adrenal glands, we selected a surgical resection of the metastatic adrenal glands. The mean overall survival time after the surgical treatment of the adrenal metastases was 23 months (7-54 months), and we considered it as a good prognosis. The mean progression free survival of the adrenal metastases was 15 months (5-30 months). Besides on such a good clinical outcome, we conclude that aggressive multimodal therapy including surgical resection of metastatic foci may be recommended if the patients with hepatocellular carcinoma have no other metastatic sites other than the adrenal gland and liver lesions are well-controlled.     

Completion lobectomy after anatomical segmentectomy

Open in a separate windowPatient selection flowchart. OBJECTIVESCompletion lobectomy (CL) after anatomical segmentectomy in the same lobe can be complicated by severe adhesions around the hilar structures and may lead to fatal bleeding and lung injury. Therefore, we aimed to investigate the perioperative outcomes of CL after anatomical segmentectomy.METHODSAmong 461 patients who underwent anatomical segmentectomy (thoracotomy, 62 patients; thoracoscopic surgery, 399 patients) between January 2005 and December 2019, data of patients who underwent CL after segmentectomy were extracted and analysed in this study.RESULTSEight patients underwent CL after segmentectomy. CL was performed via video-assisted thoracic surgery in 3 patients and thoracotomy in 5 patients. In each case, there were moderate to severe adhesions. Four patients required simultaneous resection of the pulmonary parenchyma and pulmonary artery. Thoracotomy was not required after thoracoscopic surgery in any patient. Two patients experienced complications (air leakage and arrhythmia). The median duration of hospitalization after CL was 6 (range, 5–7) days. No postoperative mortality or recurrence of lung cancer was observed. All the patients with lung cancer were alive and recurrence-free at the time of publication.CONCLUSIONSAlthough individual adhesions render surgery difficult, CL after anatomical segmentectomy shows acceptable perioperative outcomes. However, CL by video-assisted thoracoscopic surgery may be considered on a case-by-case basis depending on the initial surgery.     

Human β-defensin-3 attenuates atopic dermatitis–like inflammation through autophagy activation and the aryl hydrocarbon receptor signaling pathway

Human β-defensin-3 (hBD-3) exhibits antimicrobial and immunomodulatory activities; however, its contribution to autophagy regulation remains unclear, and the role of autophagy in the regulation of the epidermal barrier in atopic dermatitis (AD) is poorly understood. Here, keratinocyte autophagy was restrained in the skin lesions of patients with AD and murine models of AD. Interestingly, hBD-3 alleviated the IL-4– and IL-13–mediated impairment of the tight junction (TJ) barrier through keratinocyte autophagy activation, which involved aryl hydrocarbon receptor (AhR) signaling. While autophagy deficiency impaired the epidermal barrier and exacerbated inflammation, hBD-3 attenuated skin inflammation and enhanced the TJ barrier in AD. Importantly, hBD-3–mediated improvement of the TJ barrier was abolished in autophagy-deficient AD mice and in AhR-suppressed AD mice, suggesting a role for hBD-3–mediated autophagy in the regulation of the epidermal barrier and inflammation in AD. Thus, autophagy contributes to the pathogenesis of AD, and hBD-3 could be used for therapeutic purposes.     

A longitudinal analysis of paroxysmal nocturnal haemoglobinuria-type cells in patients with bone marrow failure: Results of a prospective multi-centre study in Japan

To determine the prevalence and clinical relevance of glycosylphosphatidylinositol-anchored protein-deficient (GPI[−]) cell populations (paroxysmal nocturnal haemoglobinuria [PNH]-type cells) in patients with acquired aplastic anaemia (AA) or myelodysplastic syndrome (MDS), we prospectively studied peripheral blood samples of 2402 patients (1075 with AA, 900 with MDS, 144 with PNH, and 283 with other anaemia) using a high-sensitivity flow cytometry assay in a nationwide multi-centre observational study. PNH-type cells were detected in 52.6% of AA and 13.7% of MDS patients. None of the 35 patients with refractory anaemia (RA) with ringed sideroblasts or the 86 patients with RA with excess of blasts carried PNH-type cells. Among the 317 patients possessing PNH-type granulocytes, the percentage of PNH-type granulocytes increased by ≥10% in 47 patients (14.8%), remained unchanged in 240 patients (75.7%), and decreased by ≥10% in 30 patients (9.5%) during 3 years of follow-up. PNH-type granulocyte expansion occurred more frequently (27.1%) in the 144 patients who originally carried PNH-type granulocytes ≥1% than in the 173 patients with PNH-type granulocytes <1% (4.6%). This study confirmed that PNH-type cells are undetectable in authentic clonal MDS patients, and the presence of ≥1% PNH-type granulocytes predicts a higher likelihood of PNH-type cell expansion than with <1% PNH-type granulocytes.