A 25-year-old female who had right knee and ankle joint swelling with arthralgia in July 1999, was diagnosed as having eosinophilic fasciitis (EF) based on typical skin sign and the result of muscle biopsy. Treatment was started with 20mg of oral prednisolone per day. Her symptoms had subsided but they later recurred as the prednisolone dose was gradually tapered. We measured her serum cytokine levels during the course. Her serum IFN-γ increased and IL-4 decreased after the treatment compared with those levels prior to treatment. And the cytokine levels reverted to pre-treatment status when she had a relapse. The cytokine balance seems to play some role in the etiology of EF.

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A 36-year-old man was admitted to a hospital with complaints of fever, polyarthralgia and dyspnea. Erythema was observed on his face, extensor surface of the fingers and extremities, and a chest X-ray revealed massive bilateral pleural effusion. He had no sign of myopathy at this point. Pleural fluid was proved to be exudative and contained extremely high levels of hyaluronic acid. He was also complicated with interstitial pneumonitis and was given a pulse therapy with methyl-prednisolone followed by daily administration of 55mg prednisolone (PSL). Twenty days after the commencement of the therapy, pleural effusion decreased but muscle weakness gradually appeared, accompanied by elevation of myogenic enzymes. Myogenic changes on electromyogram, and irregularity of the muscle fibers with slight inflammatory cell infiltrates in a biopsy specimen were demonstrated. He was transferred to our hospital, and a diagnosis of dermatomyositis was made. Later, pleural effusion waxed and waned depending on the dosage of PSL, but no other causative disorder was demonstrated by extensive examinations. This case indicates that the pleuritis could be one of the vasculitic manifestations of dermatomyositis.

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We report a case of a 27-year-old Japanese female with Sjögren's syndrome (SS), who suffered from several episodes of subcutaneous and mesenteric panniculitis with a recurrence within one year. After a history of fever and skin rash, the patient underwent surgery at a local hospital with a diagnosis of acute appendicitis complicated with an ileocecal abscess. She was also diagnosed as having SS. After the operation, the fever and skin rash persisted. She was treated with prednisolone (PSL), and her symptoms resolved. A recurrent bout of abdominal pain with fever, annular erythema on the trunk and a nodular erythematous rash on the lower extremities occurred six months after the operation. A skin biopsy from the lower extremities showed findings that were compatible with panniculitis. Abdominal computer tomography (CT) showed a diffuse swelling with soft tissue density in the intestinal mesenterium and para-aortic area. A retrospective examination of the operative specimen obtained from the local hospital revealed centrilobular infiltration of neutrophils in the mesenteric adipose tissue with fat necrosis, which is compatible with mesenteric panniculitis. Twenty mg/day of PSL was successful in treating the systemic panniculitis, and the abnormal diffuse soft tissue density on the abdominal CT disappeared after three weeks of PSL administration. Systemic panniculitis is a rare complication in SS, and the pathogenesis is unclear.

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Serum cytokines were evaluated in a patient with intravascular lymphomatosis (IVL). A 62-year-old man was admitted to our hospital for his general malaise, arthralgia, and highgrade fever. He was diagnosed as dermatomyositis, initially. His symptoms were improved by high dose of corticosteroid and immunosuppressive therapy. However his condition deteriorated gradually, and died ten months after the onset of symptoms. Postmortem examination revealed IVL (B cell type). The serum concentrations of inflammatory cytokines, IL-6 and TNF-α, were elevated. Level of serum IFN-γ was high only at the end of his illness. Serum IL-2 was not detected during the course. Serum IL-4 was slightly elevated. Elevated CRP and hypoalbuminemia might be related to the elevation of these inflammatory cytokines. The clinical diagnosis of IVL is usually difficult because of the absence of specific clinical manifestations and laboratory findings. The time course and/or concentrations of serum cytokines could be a key to make an early diagnosis.

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