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Tofacitinib is a novel inhibitor of Janus kinase (JAK) 3 and JAK1 is recently introduced as treatment for rheumatoid arthritis.1 The JAK inhibitors are at the focus of research in a myriad of other inflammatory diseases2 ,3 as the JAK-(signal transducer and activator of transcription) STAT pathway has a central role in cytokine signal transduction. We herein describe a case of refractory polyarteritis nodosa (PAN) successfully treated with tofacitinib.
A 28-year-old man had been diagnosed with PAN at age 14. He presented with livedo reticularis, arthritis and skin nodules with arteritis/fibrinoid necrosis confirmed on biopsy. Immunological panel at the time of diagnosis was negative for antineutrophil cytoplasmic antibodies, anti-nuclear antibodies, anti-Ro/SS-A antibodies, anti-La/SS-B antibodies, rheumatoid factor, with normal complement levels. He was treated with azathioprine and methotrexate for several years with drug-controlled complete remission. At age 24, his disease flared and he began to suffer from necrotic lesions of the scrotum and calves, excruciating abdominal pain and polyarthritis, with high C-reactive protein (CRP) levels (160–300 mg/L) for which he received recurrent intravenous methylprednisolone pulses and oral …