長嶋 孝夫

We herein report two patients with connective tissue disease who developed primary peritoneal cancer (PPC). Serum Krebs von den Lungen-6 (KL-6) levels increased when PPC was diagnosed, and these levels were correlated with the treatment and worsening of PPC in both cases. In one patient with systemic sclerosis, serum KL-6 levels increased despite stable interstitial lung disease (ILD), leading to a diagnosis of PPC. In the other patient with dermatomyositis and no ILD, PPC was diagnosed with elevated KL-6 levels four months post-treatment, without ILD development. Clinicians should be reminded that KL-6 is a tumor marker in various cancers.

A 60-year-old woman with a history of malignant lymphoma was referred to us with fever, sore throat, right-sided neck pain, and swelling while she was currently undergoing chemotherapy. Imaging studies, including computed tomography and ultrasonography of the neck, revealed significant perivascular wall thickening of the right carotid artery, which was suggestive of giant cell arteritis. However, fever, neck pain, and swelling resolved spontaneously and rapidly. Transient perivascular inflammation of the carotid artery (TIPIC) syndrome was most likely. However, these symptoms developed shortly after the administration of granulocyte colony-stimulating factor (G-CSF). Accordingly, the patient was diagnosed with G-CSF-induced aortitis mimicking TIPIC syndrome.

Cutaneous arteritis (CA) is a rare cutaneous manifestation of Crohn's disease. A 50-year-old woman with a 15-year history of CA was admitted to our hospital with a fever, abdominal pain, and hematochezia. Based on these symptoms and increased C-reactive protein levels, systemic vasculitis was considered. However, emergency colonoscopy revealed multiple longitudinal ulcers throughout the colon, thus suggesting Crohn's disease. Prednisolone (60 mg/day) was administered intravenously. A histopathological examination of the biopsied colonic mucosa revealed epithelioid granulomas, which confirmed the diagnosis of Crohn's disease. Because the systemic evolution of CA is rare, other comorbidities should be considered when patients with CA experience systemic manifestations.

A 76-year-old woman with persistent diarrhea was referred to our hospital. She had purpura, peripheral eosinophilia (18,177/μL), and an elevated serum IgG4 level (819 mg/dL). Abdominal computed tomography revealed massive ascites and bowel edema. A skin biopsy of the purpura revealed leukocytoclastic vasculitis with prominent eosinophilic infiltration. Biopsies of the gastrointestinal mucosa revealed dense eosinophilic infiltration, indicating eosinophilic gastroenteritis (EG) associated with the hypereosinophilic syndrome. The number of IgG4-positive cells increased in the duodenal mucosa; however, the diagnostic criteria for IgG4-related disease (IgG4-RD) were not met. Whether or not EG with ascites is a manifestation of IgG4-RD warrants further investigation.