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Abtract Objectives To describe the clinical, pathological, serological, and radiological characteristics of juxta-vertebral masses occurring in patients with granulomatosis with polyangiitis (GPA). Methods We analyzed the clinical records of patients with juxta-vertebral lesions from our GPA study cohort and reviewed the English literature for other cases of GPA with juxta-vertebral localization. Results Out of 74 patients in our GPA study cohort, six (8%) had juxta-vertebral lesions. We found 10 cases of juxta-vertebral GPA described in the English literature. Overall, juxta-vertebral lesions were detected at GPA onset in 11/16 (69%) patients, and preferentially occurred on the right side of the spine (12/15 patients, 80%). Fifteen patients (94%) with juxta-vertebral lesions had systemic GPA. Juxta-vertebral lesions were associated with back pain at GPA onset in 8/16 (50%) patients. In all of them juxta-vertebral lesions resolved or improved after treatment. Conclusions Preference for the right-anterior side of the spine, increased 18 FDG uptake on PET scan, low or absent invasiveness of the surrounding tissues, and occurrence in the context of systemic disease were the main features of juxta-vertebral GPA. Symptomatic lesions showed a better response to immunosuppressive therapies.

Objectives: To describe the clinical features, treatment response, and follow-up of a large cohort of Italian patients with immunoglobulin (Ig)G4-related disease (IgG4-RD) referred to a single tertiary care centre.Method: Clinical, laboratory, histological, and imaging features were retrospectively reviewed. IgG4-RD was classified as definite' or possible' according to international consensus guidelines and comprehensive diagnostic criteria for IgG4-RD. Disease activity was assessed by means of the IgG4-RD Responder Index (IgG4-RD RI).Results: Forty-one patients (15 females, 26 males) were included in this study: 26 with definite' IgG4-RD and 15 with possible' IgG4-RD. The median age at diagnosis was 62years. The median follow-up was 36 months (IQR 24-51). A history of atopy was present in 30% of patients. The pancreas, retroperitoneum, and major salivary glands were the most frequently involved organs. Serum IgG4 levels were elevated in 68% of cases. Thirty-six patients were initially treated with glucocorticoids (GCs) to induce remission. IgG4-RD RI decreased from a median of 7.8 at baseline to 2.9 after 1month of therapy. Relapse occurred in 19/41 patients (46%) and required additional immunosuppressive drugs to maintain long-term remission. Multiple flares occurred in a minority of patients. A single case of orbital pseudotumour did not respond to medical therapy and underwent surgical debulking.Conclusions: IgG4-RD is an elusive inflammatory disease to be considered in the differential diagnosis of isolated or multiple tumefactive lesions. Long-term disease control can be achieved with corticosteroids and immunosuppressive drugs in the majority of cases. ZB 0 ZR 0 ZS 0 Objectives: To describe the clinical features, treatment response, and follow-up of a large cohort of Italian patients with immunoglobulin (Ig)G4-related disease (IgG4-RD) referred to a single tertiary care centre.Method: Clinical, laboratory, histological, and imaging features were retrospectively reviewed. IgG4-RD was classified as definite' or possible' according to international consensus guidelines and comprehensive diagnostic criteria for IgG4-RD. Disease activity was assessed by means of the IgG4-RD Responder Index (IgG4-RD RI).Results: Forty-one patients (15 females, 26 males) were included in this study: 26 with definite' IgG4-RD and 15 with possible' IgG4-RD. The median age at diagnosis was 62years. The median follow-up was 36 months (IQR 24-51). A history of atopy was present in 30% of patients. The pancreas, retroperitoneum, and major salivary glands were the most frequently involved organs. Serum IgG4 levels were elevated in 68% of cases. Thirty-six patients were initially treated with glucocorticoids (GCs) to induce remission. IgG4-RD RI decreased from a median of 7.8 at baseline to 2.9 after 1month of therapy. Relapse occurred in 19/41 patients (46%) and required additional immunosuppressive drugs to maintain long-term remission. Multiple flares occurred in a minority of patients. A single case of orbital pseudotumour did not respond to medical therapy and underwent surgical debulking.Conclusions: IgG4-RD is an elusive inflammatory disease to be considered in the differential diagnosis of isolated or multiple tumefactive lesions. Long-term disease control can be achieved with corticosteroids and immunosuppressive drugs in the majority of cases.

We describe an immunocompetent 61-year-old woman who was negative for human immunodeficiency virus and who had recurrent human herpesvirus 8 (HHV-8) infection associated with a relapsing systemic inflammatory syndrome characterized by fever, lymphadenopathy, splenomegaly, edema, arthrosynovitis, and rash. Kaposi's sarcoma developed 10 months after the initial clinical presentation. A correlation was documented between the recurrent clinical manifestations and the HHV-8 load in plasma and peripheral-blood mononuclear cells. Histologic examination of an enlarged lymph node heavily infected with HHV-8 revealed an atypical lymphoproliferative disorder characterized by paracortical hyperplasia and collapsed primary and secondary follicles.

Giant cell arteritis (GCA) rapidly responds to high-dose corticosteroids. However, smouldering arterial inflammation can persist despite the absence of symptoms and altered acute phase reactants. In patients that are refractory, symptoms relapse during steroid tapering and vascular complications may develop. The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level are not sensitive enough markers to detect refractory disease.1 The neuroendocrine system regulates innate and acquired immune responses, influencing cytokine synthesis and limiting tissue damage via release of neurotransmitters and peptides in peripheral tissues. Chromogranin A in particular is a candidate marker linking neurogenic inflammation and vascular inflammation.2 We investigated by ELISA, as described previously,3 the …

Objective To verify whether the prototypical long pentraxin PTX3 represents an indicator of the activity of small-vessel vasculitis. Methods Concentrations of PTX3, a pentraxin induced in endothelium by cytokines, were measured by enzyme-linked immunosorbent assay in the sera of 43 patients with Churg-Strauss syndrome, Wegener's granulomatosis, and microscopic polyangiitis. PTX3 was also measured in the sera of 28 patients with systemic lupus erythematosus (SLE), 22 with rheumatoid arthritis, and 16 with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias). Serum concentrations of C-reactive protein (CRP) were measured by immunoturbidimetry. The cells involved in PTX3 production in vivo were identified in skin biopsy samples. Results Patients with active vasculitis had significantly higher concentrations of PTX3 than did those with quiescent disease (P < 0.001). PTX3 levels in the latter group were similar to those in healthy controls. PTX3 levels were higher in patients with untreated vasculitis and lower in patients who underwent immunosuppressive treatments (P < 0.005). In contrast, patients with active SLE had negligible levels of the pentraxin. PTX3 levels did not correlate with CRP levels in vasculitis patients. Endothelial cells produced PTX3 in active skin lesions. Conclusion PTX3 represents a novel acute-phase reactant produced at sites of active vasculitis.

Charcot’s arthropathy is a degenerative condition of the joints secondary to a neurologic lesion, resulting in deformity and increased bone reabsorption1. Misdiagnosis as osteomyelitis or neoplasm is frequent. A 67-year-old man with a 19-year history of posttraumatic sensorimotor paraplegia, depression, and alcoholism was evaluated for weight loss of 4 kg over 2 months. At examination, he was depressed, malnourished, and emaciated. Sensorimotor paraplegia …

Hypoglycaemia associated with lactic acidosis is a rare complication of lymphomas; only four cases have been previously reported. Recent studies provide evidence of direct consumption of glucose by the tumour cells, leading to lactic acidosis. We report the case of a 64-year-old patient with a gastric diffuse large B cell non-Hodgkin's lymphoma transformed from an indolent mucosa associated lymphoid tissue (MALT) lymphoma, admitted to our department for acute renal failure due to a tumour lysis syndrome. After recovery from renal failure, she developed severe hypoglycaemia and lactic acidosis refractory to therapy. She died after the onset of shock and coma.

This is the first reported case of a young patient with ulcerative colitis who developed acute Budd-Chiari syndrome and was found positive for anticardiolipin antibodies. Although an association between ulcerative colitis and Budd-Chiari syndrome, and between the latter and antiphospholipid antibodies, has been shown in the literature, the coexistence of these three conditions has never been reported. The patient we describe did poorly and was considered as a candidate for liver transplantation in spite of prompt heparin therapy, probably because of the presence of multiple risk factors.

Eight patients with stable Behçet's disease were studied by means of multimodality evoked potentials and magnetic resonance imaging to evaluate the possibility of an earlier and objective demonstration of clinical and subclinical Central Nervous System (CNS) involvement. It was shown that both diagnostic techniques are useful for quantitative evaluation of neurological involvement in Behçet's disease; of particular interest was the demonstration of subclinical CNS changes.