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A 68-year-old man was admitted because of weakness of the left leg, dysesthesiae of the extremities and bilateral lower extremity purpura. A neurological examination showed mononeuritis multiplex with laboratory evidence of hypocomplementemia, cryoglobulinemia and leukocytoclastic vasculitis in the biopsy of a skin specimen. The patient also exhibited bilateral submandibular gland swelling, elevated serum IgG4 levels and infiltration of a large number of IgG4-positive plasma cells in the submandibular glands. These findings were consistent with both cryoglobulinemic vasculitis and IgG4-related disease. The administration of oral prednisolone (1 mg/kg/day) resolved the neurological manifestations and the swelling of the submandibular glands and cryoglobulinemia.

We report the findings of a 46-year-old man, who presented with fever and renal dysfunction while undergoing treatment for Crohn's disease with infliximab (IFX). Remittent fever and renal dysfunction with urinary casts developed and lasted for 3 weeks without deterioration of Crohn's disease. Renal biopsy revealed acute tubulointerstitial nephritis (ATIN). After the discontinuation of IFX, his fever and renal abnormalities resolved. We herein report the first known case of ATIN associated with IFX.

A 30-year-old woman had suffered from recurrent and self-limiting fevers since childhood. Although she had no mutations in the exons or introns of the tumor necrosis factor (TNF) receptor superfamily member 1A gene, her clinical characteristics were consistent with those of TNF receptor-associated periodic syndrome (TRAPS). She did not respond to treatment with etanercept, although tocilizumab therapy was successful, subsequently ameliorating her symptoms and preventing further inflammatory attacks. Interleukin-6 blocking therapy should be considered as a new alternative treatment in patients with TRAPS who do not respond to etanercept.

A 20-year-old woman who had suffered from Still's disease was admitted for fever and progressive pneumonitis after long-term remission. High spiking fever, leukocytosis, splenomegaly and an extremely high serum ferritin concentration strongly suggested a relapse of Still's disease. Intensive therapy with high-dose methylprednisolone, cyclophosphamide and gamma globulin was required for the severe pneumonitis, which was thought to be a rare manifestation in Still's disease.(Internal Medicine 32: 902-905, 1993)

Clinical and pathological features of eight fatal cases with Sjogren's syndrome (all female, aged 43-61) were studied from the prognostic viewpoint. Chronic active hepatitis, primary biliary cirrhosis, diffuse interstitial pneumonitis and primary pulmonary hypertension were fatal illness in seven of them, and remaining one died of accidental infection. The clinical and pathological findings of these cases suggested that such hepatic or pulmonary diseases might be the manifestations of Sjogren's syndrome. Interstitial nephritis mayalso be a manifestation of the syndrome, possibly with a fatal outcome. Fromthese observations Sjogren's syndrome with liver, lung or kidney involvement may be a possible clinical type with poor prognosis.