Because type I IFN plays an important part in the pathogenesis of SAVI, it is highly likely that JAK inhibitors are an effective treatment, and JAK inhibitors deserve further thought as treatment options in such individuals. established treatment protocol for SAVI. However, based on its pathogenesis, Janus kinase (JAK) inhibitors are expected to be effective. In fact, a small number of studies have reported the effectiveness of this treatment (3,12). We herein statement a patient who Vwf developed atypical pulmonary lesions during treatment for juvenile idiopathic arthritis (JIA) and was ultimately diagnosed with SAVI. Case Statement An 18-year-old Japanese man visited our hospital for respiratory distress and joint pain. At 2 years old (X-16), the patient had swelling, pain, and limited range of motion of the hand, knee, and foot bones and been examined at a local orthopedic medical center. X-ray imaging experienced demonstrated no abnormalities, and he had been adopted up without treatment. However, his joint-related symptoms persisted. In X-13, he had contracture of bilateral hand joints, pain in the remaining shoulder joint, and neck pain. Concurrently, he also developed dyspnea, and in X-12, he was admitted to the Division of Pediatrics at our hospital for a detailed examination. Plain chest X-ray and computed tomography (CT) showed significant interstitial pneumonia, and his Krebs von den Lungen (KL)-6 level experienced significantly increased to 2,743 U/mL. Based on the prolonged multiple joint symptoms, the patient was diagnosed with JIA associated with interstitial pneumonia, and treatment was started with glucocorticoid (GC) and methotrexate (MTX). The joint symptoms resolved with the treatment, but the interstitial changes in the lungs gradually progressed. In X-11, he was found to have a synovial cyst within the dorsum of the hand and was suspected of having early-onset sarcoidosis (EOS). However, granulomas were not observed, and genetic testing showed no mutations associated with EOS. In X-7, combination therapy with azathioprine was started, and GC pulse therapy was also given. However, his interstitial pneumonia continued to progress, and general malaise and multiple joint pain were exacerbated from the reduction in the dose of GC. In X-5, the tumor necrosis element (TNF) inhibitor adalimumab was started but discontinued after several months because of an increase in the KL-6 level and its overall ineffectiveness. As with the earlier treatment, the emphysematous changes in the lungs continued to progress gradually, and the joint symptoms were exacerbated from the reduction in the dose of GC. Consequently, in April of X-2, the patient was referred to our division for a further assessment of the analysis (Fig. 1, Fig. 2A, B). Doxifluridine Open in a separate window Number 1. Clinical history until referral Doxifluridine to our division. JIA: juvenile idiopathic arthritis, ADA: adalimumab, PSL: prednisolone, mPSL: methylprednisolone, MTX: methotrexate, AZ: azathioprine Open in a Doxifluridine separate window Number 2. Plain chest computed tomography findings. A) December of X-13; B) August of X-7; C) April of X-2; and D) June of X. Progression of interstitial pneumonia and emphysematous changes are seen. In X-2 (the time of his 1st admission to our division), his height was 140.6 cm, weight was 36.3 kg, body temperature was 36.4 C, and percutaneous oxygen saturation (SpO2) was 95% (space air). Chest auscultation revealed good crackles in the bilateral middle-lower lung fields. There was no tenderness, joint swelling, or rash. A blood count showed an elevated white blood cell count. There was no elevation of Doxifluridine hepatobiliary enzymes, and his renal function was normal. The C-reactive protein (CRP) level was slightly elevated. KL-6 was markedly elevated to 4,597 U/mL. The immunoglobulin (Ig)G level was improved, and the antinuclear antibody titer was 1:320 (homogeneous pattern). However, no disease-specific autoantibodies were found. In addition, myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) and proteinase (PR)3-ANCA titers were elevated (Table 1). X-ray of the hand and foot bones showed no joint space.
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