Because the case of combined pulmonary and osseous cryptococcosis is rarer, we decided to report it. Neurological involvement in Sj?grens syndrome can be manifested as peripheral neuropathy and/or demyelinating encephalopathy, which is rare and highly malignant, and the pathology may be vasculitis [2], as in the patient we reported, whose cranial MRI displayed symmetrical demyelinating lesions according to the vascular distribution. of bilateral cerebral hemispheres. Electromyography indicated severe peripheral nerve injury, especially in lower limbs. Computed tomography scan of lumbar vertebral displayed multiple high-density shadows, and the corresponding vertebrae on magnetic resonance imaging showed abnormal low transmission intensity on T1 and T2 sequences. Positron emission tomographyCcomputed tomography showed multiple lesions with high Avanafil 18F-fluorodeoxyglucose uptake in lung and vertebral body. Both lung and bone biopsies Avanafil suggested contamination, with the diagnosis of Sj?grens syndrome with nervous system injury combined pulmonary and osseous cryptococcosis. She took a reduced dose of prednisone about 10?mg/day, terminated mycophenolate mofetil, and began to take immunoglobulin of 0.4?g/kg/day intravenously for 5?days, fluconazole (400?mg/day) for 6?months. Within 3?weeks, her chest radiography showed a marked improvement, and 3?months later, the pulmonary lesions disappeared on her computed tomography scan. Conclusions This case exhibits an extremely rare condition of neural involvement in Sj? grens syndrome combined with pulmonary and osseous cryptococcosis. This statement also highlights the crucial role of detailed clinical examination, serologic markers, and biopsy in avoiding misdiagnosis. Currently, there is no guideline for this situation; in this case, we controlled the disease successfully with antifungal drugs and adequate gamma globulin, followed by an appropriate dose of corticosteroids. and positive ink staining (Fig?(Fig.4).4) and negative acid-fast staining, which was consistent with contamination, and no were found in the cerebrospinal fluid (CSF) . Open GLB1 in a separate windows Fig. 1 Cranial MRI exposing multiple demyelinating lesions in the white matter of both cerebral hemispheres Open in a separate windows Fig. 2 a CT scan showing multiple high-density lesions in lumbar vertebrae. b, c MRI demonstrating abnormal low transmission in the corresponding vertebral body at T1 and T2 sequences. Red arrows point to the lesions Open in a separate windows Fig. 3 PET/CT showing multiple lesions with high 18F-FDG uptake in lung (a) and vertebral body (b) Open in a separate windows Fig. 4 a Lung tissue biopsy showing granulomatous lesions [hematoxylin and eosin (HE), 100]. b Lung tissue biopsy showing granulomatous lesions; green arrow points to [periodic acidCSchiff (PAS), 400]. c Positive ink staining Open in a separate windows Fig. 5 a CT scan showing multiple nodules in the left lung. b CT scan showing a reduction in lung lesions. Red arrows point to the lesions She was eventually diagnosed with neurologic complications related to Sj?grens syndrome combined with pulmonary and osseous cryptococcosis. The dose of prednisone was reduced to 10?mg/day, MMF was discontinued, and fluconazole (400?mg/day) was given for 6?months. In the mean time, to modulate the immunity, immunoglobulin of 0.4?g/kg/day was injected intravenously for 5?days. Her CT scan showed that this lung lesions experienced reduced within 3?weeks (Fig?(Fig.5b),.5b), and 3?months later, these lesions had disappeared. However, during this period, the symptoms of limb numbness worsened, indicating that SS was active. Even though contamination was not Avanafil fully controlled, she received therapy with prednisone (30?mg/day) since the 45th day after the treatment of fluconazole. Fortunately, her neurological symptoms and the contamination were both controlled during the recent 1-12 months follow-up period. Conversation and conclusion SS is usually a rare syndrome characterized by dry mouth and eyes, and 10C60% patients of suffer nervous system damage, with 2C25% in Avanafil central nervous system [1]. Only a few cases of central and peripheral nervous system damage simultaneously in SS have been reported. Because the case of combined pulmonary and osseous cryptococcosis is usually rarer, we decided to statement it. Neurological involvement in Sj?grens syndrome can be manifested as peripheral neuropathy and/or demyelinating encephalopathy, which is rare and highly malignant, and the pathology may be vasculitis [2], as in the patient we reported, whose cranial MRI displayed symmetrical demyelinating lesions according to the vascular distribution. In the mean time, it has been reported that, in some patients with intracranial lesions, distribution of blood vessels is not consistent, showing multiple asymmetries, and it is speculated that this pathology may also be related to lymphocyte infiltration in the central nervous system [3]. The severity of the central nervous system lesions in SS is usually associated with anti-SSA antibody, but not with ANA or anti-SSB antibody?[4], as in this case. Clinical manifestations of SS nervous system involvement are varied, repeatable, and multifocal, bearing a close resemblance to multiple sclerosis in symptoms, CSF, and imaging, but.