We report a 35-year-old

Objectives Hypertrophic pachymeningitis (HP) is a rare complication in patients with anti-neutrophil antibody-associated vasculitis (AAV); its clinical features, incidence, and risk factors remain unknown. We aimed to clarify the prevalence, clinical features, and factors associated with new-onset HP in patients with AAV. Method A retrospective cohort study involving 93 patients with AAV was conducted. HP incidence between patients with granulomatosis with polyangiitis (GPA) and those with microscopic polyangiitis (MPA) was compared to investigate risk factors associated with HP. We performed only univariate analysis using logistic regression and classification tree (CART) analysis due to the small number of HP cases. Results Among the 93 patients (76 with MPA and 17 with GPA), only 6 patients developed HP (1 with MPA, 5 with GPA) over an average observation period of 4 years; all patients who developed HP were positive for myeloperoxidase anti-neutrophil antibody. HP incidence was significantly higher in patients with GPA than in those with MPA (60.2 versus 3.3 persons per 1000 person-years, respectively, P = 0.002). The univariate analysis revealed that otitis media (P < 0.001) and sinusitis (P = 0.014) were associated with new-onset HP. Univariate CART analysis grouped the patients into patients with HP who have otitis media (33%) and patients with HP who have sinusitis (21%). The odds ratio of otitis media adjusted by age and first diagnosis of AAV was 38.1 (95% confidence interval, 3.08-331.4; P = 0.004). Conclusions Although only in the univariate analysis, otitis media was the most discriminating factor to predict new-onset HP in patients with AAV.

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Introduction Currently, there are no reports of diaphyseal femoral fracture equivalent to atypical femoral fractures (AFFs) in patients receiving long-term hemodialysis (HD). Case report A 56-year-old Japanese man receiving long-term HD for 34 years was admitted to our hospital due to a delay in postoperative healing. The patient began maintenance hemodialysis at 22 years of age. The patient then underwent surgical parathyroidectomy (PTX) for secondary hyperparathyroidism at 43 years of age, which resulted in decreased levels of parathyroid hormone (PTH). Thereafter, this patient’s serum 1,25(OH)2 D3 level was very low because active vitamin D3 derivative was not administered. At 54 years of age, a transverse fracture of the femoral shaft equivalent to AFF occurred. Surgery with open reduction and internal fixation using intramedullary nailing was performed; however, the delay of postoperative healing continued for 16 months. A left iliac crest bone biopsy was performed and showed osteoid-like lesion and an increase of woven bone. The patient received active vitamin D3 derivative and recombinant human PTH (1–34) derivative. Twenty-nine months after the first surgery, a reoperation was performed. Simultaneously, a right iliac crest bone biopsy was performed. Bone morphometrical improvement was confirmed. Six months after resurgery, the bone union was achieved. Summary Severe vitamin D3 deficiency and decreased levels of PTH may induce a higher osteoid state and an increase of woven bone, which may then attribute to the development of diaphyseal femoral fracture and impairment of postoperative bone healing. It is hypothesized that treatment with active vitamin D3 and teriparatide acetate may be a therapeutic option via the accelerated formation of lamellar bone for refractory diaphyseal femoral fracture of long-term dialysis.

A 65-year-old woman with a limited form of systematic sclerosis (SSc) and Sjögren's syndrome (SS) was admitted to our hospital for the evaluation of renal dysfunction. Her serum creatinine was 1.6 mg/dl, proteinuria was 1.6 g/day, and the urine sediment contained 20-29 erythrocytes/high-power field. Myeloperoxidase anti-neutrophil cytoplasmic antibodies, anti-SS-A/SS-B antibodies and anti-centromere antibodies were positive. A renal biopsy showed focal necrotizing glomerulonephritis with focal interstitial lymphoplasmacytic infiltration. A diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) was made. A steroid therapy was initiated and AAV subsided. This is a rare case of AAV in a patient with anti-centromere-positive limited SSc and SS.