The Efficacy of Baricitinib in Real-World Patients with Refractory or Severe Juvenile Dermatomyositis:A Monocentric Retrospective Study

Objective To evaluate the efficacy and safety of low dose baricitinib in children with refractory or severe juvenile dermatomyositis (JDM) in a real-world setting. Methods A monocentric retrospective real-world study was conducted, in which fourteen refractory and one severe newly diagnosed JDM patients were included. These patients were all treated by low dose baricitinib (below the recommended dose) combined with corticosteroids and or immunosuppressive agents. Clinical data were collected at the baseline and 4, 12, 24 weeks after baricitinib implication. Treatment response (complete response (CR), Partial response (PR) and non-response (NR)) was evaluated using both the Paediatric Rheumatology International Trials Organization (PRINTO) remission criteria and skin Disease Activity Score (DAS). All the adverse events (AEs) were recorded. Results After baricitinib treatment, all 15 patients showed improvement of skin involvement, including 14 patients with recurrent skin rashes and one newly diagnosed JDM. Calcinosis stabilized in two patients (2/3) and partially regressed in one. Four patients (4/15) had interstitial lung disease (ILD), which normalized in one, improved in two and stabilized in one. One patient complicated with macrophage activation syndrome (MAS) achieved clinical remission. CR was achieved in 3/15 patients, ranging from 4 to 12 weeks after baricitinib initiation. Five patients (5/15) got PR 4 to 24 weeks after baricitinib use. Daily steroid dosage was decreased from 0.632 mg/kg to 0.357 mg/kg (P = 0.043) at 24 weeks in all responders. However, there was no statistically difference in muscle improvement. One patient was stopped using baricitinib because of varicella zoster virus infection, while no other serious side effect was observed in this study. Conclusion Low dose baricitinib had efficacy and was safe to applied in refractory or severe JDM patients, especially for recurrent skin rashes. Baricitinib may also be helpful for JDM complicated with ILD and MAS.

Severe Hemorrhage Secondary To IgA Vasculitis With Varicella Infection: A Case Report

IgA vasculitis (IgAV), formerly named as Henoch Schönlein Purpura, is an IgA-mediated systemic small vessel vasculitis with skin, gastrointestinal tract, joints, and kidneys involvement. It is the most common vasculitis in childhood. Varicella-zoster virus-associated IgA vasculitis has been reported previously in few cases. However, IgA vasculitis complicated by chickenpox rarely occurred severe hemorrhagic manifestation and had not yet been reported. Herein, we present a case of an eight-year-old girl who developed IgA vasculitis complicated by disseminated intravascular coagulation and multiple organ dysfunction syndrome, including severe acute pancreatitis and acute liver failure secondary to varicella-zoster virus infection and finally underwent blood purification including hemoperfusion, plasmapheresis, and continuous renal replacement therapy. Tumors and other diseases affecting coagulation and platelet function, such as systemic lupus erythematosus, ANCA-associated vasculitis, primary thrombocytopenia, and secondary thrombotic microangiopathies, have been excluded during her hospitalization. Varicella was considered to be the cause of the severe complications. A possible mechanism for the occurrence of severe complications is that chickenpox infection induces the production of heparin-like substances disturbing normal coagulation function, and finally occurs uncontrolled hemorrhage manifestation including gastrointestinal hemorrhage, uncontrolled bleeding at the catheter placement and injection site. She eventually was discharged after undergoing 37 days of treatment by antibiotics, antiviral drugs, protamine neutralizing heparin therapy and various blood products transfusion including platelets, red cells, fresh frozen plasma, cryoprecipitate, fibrinogen, and prothrombin complex transfusions. At the end of a month follow-up, the patient showed the purpuric rashes had nearly faded and had no sign of complication including the gastrointestinal tract, liver, and pancreas. Renal function tests and routine analysis of blood were normal. Hematuria(3+~4+) and proteinuria(1+~2+) can be seen in her urinalyses. At the 5th month follow-up, her renal function test and her urinalysis were normal, and her oral steroids were stopped completely. On this occasion, we should reduce the dose of heparin or no heparin in blood purification for IgA vasculitis complicated by chickenpox to reduce the incidence of severe bleeding or death.

Ramsay hunt syndrome- expect the unexpected: A case report

Herpes zoster represents a latent reactivation of the varicella zoster virus infection with a dermatomal pattern of eruption and complicated by post-herpetic neuralgia. Ramsay hunt syndrome is a rare complication of herpes zoster and it is not just a syndrome but it's rather an infectious disease. This syndrome is characterized by peripheral facial nerve palsy associated with an erythematous vesicular rash on the ear. It is characterized by unilateral pattern of facial involvement and presence of vesicles helps in early diagnosis and distinguish the syndrome with diseases mimicking other severe neurological diseases. This article reports a case of 56-year-old male patient who reported with a complaint of severe toothache, which serves as severe prodrome for reactivation of herpes zoster virus which later leads to Ramsay Hunt syndrome. This case report highlights about the management of herpes zoster and its complication and emphasizes on prevention of post herpetic neuralgia complication.

Ramsay hunt syndrome- expect the unexpected: A case report

Herpes zoster represents a latent reactivation of the varicella zoster virus infection with a dermatomal pattern of eruption and complicated by post-herpetic neuralgia. Ramsay hunt syndrome is a rare complication of herpes zoster and it is not just a syndrome but it's rather an infectious disease. This syndrome is characterized by peripheral facial nerve palsy associated with an erythematous vesicular rash on the ear. It is characterized by unilateral pattern of facial involvement and presence of vesicles helps in early diagnosis and distinguish the syndrome with diseases mimicking other severe neurological diseases. This article reports a case of 56-year-old male patient who reported with a complaint of severe toothache, which serves as severe prodrome for reactivation of herpes zoster virus which later leads to Ramsay Hunt syndrome. This case report highlights about the management of herpes zoster and its complication and emphasizes on prevention of post herpetic neuralgia complication.