Search

Testicular vasculitis (TV) develops when an organ is involved in systemic vasculitis. A 47-year-old man with eosinophilic granulomatosis with polyangiitis (EGPA) developed TV as the first clinical episode. The patient had bronchial asthma for 8 years and developed left testicular pain before developing arthralgia, abdominal involvement, and sensory polyneuropathy, which led to the diagnosis of EGPA. The induration of the affected testicle persisted even after initiating immunosuppressive therapy with corticosteroids and cyclophosphamide, raising concern for testicular neoplasm, while testicular pain and other symptoms resolved. The patient underwent inguinal orchiectomy, and a histology examination of the resected testicle revealed fibrinoid necrotizing vasculitis. Only three cases of biopsy-proven TV in patients with EGPA have been reported in our review of published English-language articles. Two of the three patients in the reviewed cases developed TV before being diagnosed with EGPA. Moreover, all patients underwent extirpation of the affected testicle, leading to a pathological diagnosis of TV. This report suggests that TV may develop and be the presenting condition in EGPA, although urogenital involvement is rare., (© 2022. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Hypertrophic pachymeningitis (HP) is a rare neurological disorder with focal or diffuse thickening of the dura mater, which usually causes headache, cranial neuropathies, seizures, and motor or sensory impairments. The development of HP is attributed to an immune-mediated mechanism, and some autoimmune diseases have been implicated in the development of HP. Herein, we describe the case of a 73-year-old woman with persistent headache ascribable to HP, which developed approximately 3 years after a diagnosis of polyarteritis nodosa (PAN). She was treated with high-dose corticosteroid and cyclophosphamide that resulted in immediate disappearance of headache and improved radiological findings of thickened dura mater. In addition, she was subsequently administered methotrexate, ultimately resulting in maintenance of remission and regular reduction of prednisolone. In our review of published English articles, only two cases of HP in patients with PAN have been reported to date, suggesting that HP is a rare complication in PAN. However, it should be recognized that HP may develop as a neurological involvement related to PAN., (© 2021. International League of Associations for Rheumatology (ILAR).)

Moyamoya syndrome (MMS) is a chronic cerebrovascular disorder characterized by occlusion or stenosis of the internal carotid arteries with the formation of abnormal collateral vascular networks. Moreover, the development of MMS, which is a distinct category from "moyamoya disease," is attributed to the underlying disease, while some cases of MMS related to systemic lupus erythematosus (SLE) have been previously reported. Herein, we present the case of a 29-year-old Japanese woman with SLE in whom intracranial hemorrhage ascribable to MMS developed during pregnancy. Craniotomy was performed to remove hematoma, and prednisolone, tacrolimus, and hydroxychloroquine were consecutively administered. She ultimately achieved remission and childbearing without the relapse of cerebrovascular event. To our knowledge, this is the first report of MMS associated with SLE in pregnancy. Through reviewing published English articles and our case, it was suggested that the pathogenesis of SLE is implicated in the development of moyamoya vasculopathy leading to cerebrovascular events. Moreover, pregnancy may affect the bleeding from the fragile collateral vessel wall.

Objective: Hypertrophic pachymeningitis (HP) related to anti-neutrophil cytoplasmic antibody (ANCA) is the most frequently seen immune-mediated HP. We investigated cerebrospinal fluid (CSF) biomarkers related to the pathogenesis of ANCA-related HP (ANCA-HP)., Methods: The levels of B cell activation factor of the tumor necrosis factor family (BAFF), a proliferation-inducing ligand (APRIL), and transforming growth factor beta 1 (TGF-β1) in the CSF were compared between patients with ANCA-HP (n = 12), other types of immune-mediated HP (other HP; n = 12), multiple sclerosis (MS; n = 14), and non-inflammatory neurological disorders (NIND; n = 10). In addition, we evaluated whether ANCA would be detected in CSF., Results: CSF levels of BAFF, APRIL, and TGF-β1 were significantly increased in ANCA-HP and other HP. In particular, BAFF and APRIL levels were significantly correlated with the IgG index in ANCA-HP. In other HP, BAFF and APRIL levels were significantly correlated with cell counts and protein levels in CSF. Of 12 patients with ANCA-HP, the CSF of 7 patients (58%) tested positive for myeloperoxidase (MPO)- or proteinase 3 (PR3)-ANCA, while none of the CSF samples from other HP, MS, or NIND patients tested positive., Conclusion: The levels of BAFF, APRIL, and TGF-β1 may serve as useful CSF biomarkers for assessing the disease activity of immune-mediated HP. Moreover, BAFF and APRIL in the CSF may be implicated in the pathogenesis of ANCA-HP via promoting autoreactive B cells, while detecting MPO- or PR3-ANCA in the CSF may be found in some patients with ANCA-HP.Key Points• CSF BAFF, APRIL, and TGF-β1 levels increase significantly in immune-mediated HP.• CSF BAFF and APRIL levels are significantly correlated with IgG index in ANCA-HP.• Detection of MPO- or PR3-ANCA in the CSF is found in some patients with ANCA-HP.• BAFF, APRIL, and ANCA in the CSF may be implicated in the pathogenesis of ANCA-HP.