ABSTRACT Objective To revise the 2017 clinical practice guidelines (CPG) for the management of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) to reflect advancements in the field. Methods Similar to the 2017 CPG, the Grading of Recommendations, Assessment, Development, and Evaluation system was adopted for this revision. The intended users of this CPG include patients diagnosed with MPA or GPA in Japan and their families and healthcare professionals, including specialists and non-specialists. Based on a scoping review, four clinical questions (CQs) of the 2017 guidelines were modified, and six new CQs were added. Results We suggest a combination of glucocorticoid and cyclophosphamide or rituximab for remission induction therapy. In cases where cyclophosphamide or rituximab is used, we suggest the use of avacopan over high-dose glucocorticoid. Furthermore, we suggest against the use of plasma exchange in addition to the standard treatment in severe cases of MPA/GPA. Finally, we suggest the use of glucocorticoid and rituximab over glucocorticoid and azathioprine for remission maintenance therapy. Conclusions The recommendations have been updated based on patient preference, certainty of evidence, benefit and risk balance, and cost.
Meningioma is a common intracranial tumor, accounting for 13-18% of all intracranial tumors, however, extracranial meningioma is very rare, especially with extension into the temporal bone. A 49-year-old female had complained of left hearing loss for several years. A granuloma-like mass was seen through the left tympanic membrane, and a pure tone audiogram showed left combined hearing loss. We performed left tympanoplasty with mastoidectomy. At the time of operation, the granuloma-like mass was seen in the mastoid and tympanic cavities, postoperative pathological study revealed it to be a meningioma. After the operation, CT and MRI scans showed a large mass in the C-P angle. We conclude that CT and MRI examinations are valuable when there is the possibility of a tumor.
We report a rare case of papillary thyroid carcinoma with pathological fracture. A 69-year-old male reporting left femoral pain was found in pathological orthopedic examination to have papillary thyroid carcinoma metastasis to the left femur but no apparent distant metastasis, necessitating total thyroidectomy and radioiodine therapy twice, but thyroglobulin remained elevated. To preserve all possible quality of life, we continued radiation therapy as the thyroid cancer spread to multiple bones and liver sites.
Epithelial-myoepithelial carcinoma (EMCa) comprises less than 1% of all salivary gland neoplasms and mainly involves the major salivary glands, especially the parotid gland. We report on a case of an EMCa arising from the parotid gland. An 86-year-old woman visited our hospital with a painless mass in the left subaural region which had been present for two years. Head and neck imagings revealed a 28×30 mm mass in the left parotid gland. There was no apparent facial nerve involvement on clinical evaluation. A pleomorphic adenoma was suspected following cytodiagnosis of a needle biopsy specimen. The tumor was resected with the left superficial part of the parotid gland on November 6, 2006. Histopathological and immunohistochemical examination revealed that the inner cells had differentiated to the ductal epithelium and outer cells to the myoepithelium. Consequently, the final diagnosis was an epithelial-myoepithelial carcinoma. Two years after surgery, the patient’s postoperative course has been uneventful.
Squamous cell carcinoma of the nasal cavity and paranasal sinuses is comparatively common, while nonsquamous cell carcinoma—adenocarcinoma and salivary gland-type carcinoma—is rare. We examined reports in Japanese about nonsquamous cell carcinoma of the nasal cavity and paranasal sinus after 2000 finding adenoid cystic carcinoma to be most common followed by adenocarcinoma, and mucoepidermoid carcinoma. We retrospectively analyzed 8 patients (2 men and 6 women, median age: 67.5 years) with salivary carcinoma of the nasal cavity and paranasal sinuses between 1990 and 2009. Primary sites were the maxillary sinus in 4, ethmoidal sinus in 2, and nasal cavity in 2. Pathology included adenocarcinoma in 4, adenoid cystic carcinoma in 3, and mucoepidermoid carcinoma in 1. T classification was T2 in 3, T3 in 3, and T4a in 2. No lymph node or remote metastases were seen. Stage II accounted for 3, stage III for 3, and stage IVA for 2. Surgery was done in all 8 cases-surgery alone in 3, surgery and radiotherapy in 4, and 1 with adenoid cystic carcinoma by combined surgery, radiotherapy, and chemotherapy. None of the 8 showed postoperative recurrences or metastasis, and all survived primary treatment.
Pustulosis palmaris et plantaris (PPP) and pustulotic arthro-osteitis (PAO) are tonsil-related diseases. Treatment outcome of tonsillectomy and prognostic factors influencing the outcome have not been analyzed quantitatively. We evaluated those using the Palmoplantar Pustulosis Area and Severity Index (PPPASI). At 1, 3, 6, 12, 24 and more than 24 months post-tonsillectomy, 20 (31%), 34 (48%), 70 (60%), 57 (80%), 36 (95%) and 23 (96%) patients realized 80% or more improvement of PPP skin lesions, respectively, and eight (17%), 23 (36%), 30 (50%), 38 (79%), 12 (100%) and four (100%) patients showed 80% or more improvement of PPPASI (i.e. PPPASI% ≥ 80%), respectively. At 1, 3, 6, 12 and more than 12 months post-tonsillectomy, 19 (73%), 21 (66%), 27 (73%), 19 (79%) and 15 (83%) patients realized a disappearance of PAO-induced arthralgia, respectively. Kaplan-Meier analysis of 80 patients with PPP revealed that, at 12 and 24 months post-tonsillectomy, lesions disappeared (i.e. PPPASI = 0) in 38% and 66% of patients, respectively, and lesions improved by 80% or more (i.e. PPPASI% ≥ 80%) in 71% and 95% of patients, respectively. The log-rank test and univariate and multivariate analyses showed that smoking cessation post-tonsillectomy and PAO were significant predictive factors for the early disappearance of skin lesions. This report is the first demonstrating objective evidence of the great efficacy of tonsillectomy to improve PPP skin lesions. Even post-tonsillectomy, smoking inhibited the early disappearance of the lesions.
