Rapidly progressive interstitial lung disease in the course of juvenile dermatomyositis: a case report

Abstract

Abstract Background Juvenile dermatomyositis is a systemic, autoimmune inflammatory muscle disorder and vasculopathy. While generally it is well-responsive to the standard line of therapy, in rare cases may develop life-threatening complications and show resistance to treatment. We presented the case of a 7-year-old, who developed rapidly progressive interstitial lung disease and pancreatic dysfunction in the course of fulminant juvenile dermatomyositis with a good outcome after aggressive immunosuppressive therapy with the use of rituximab. Our case illustrates the diagnostic challenges of this condition in the setting of non-specific clinical manifestations and a need for novel molecular mechanism-based treatment to improve the survival of refractory dermatomyositis. Case presentation A 7-year-old girl presented with erythematous rash, muscle weakness, pain of lower extremities and elevated body temperature. The patient was diagnosed with juvenile dermatomyositis through physical examination (typical skin manifestation, proximal muscle weakness) and based on the finding of elevated levels of serum muscle enzymes and myopathic EMG changes. In the course of the disease she developed interstitial lung disease rapidly progressing to respiratory dysfunction and pancreatic failure. She received combined therapy, including methylprednisolone pulse therapy, followed by intravenous immunoglobulin, both in maximum doses per kilogram. On the 20th day of hospitalization, she was admitted to the intensive care unit and placed on ventilation support. On the 11th day of assisted ventilation rituximab was administered. Because of pancreatic dysfunction, complicating the course of the disease, the child was placed on total parenteral nutrition. After 3 doses of rituximab her general state and respiratory condition showed gradual improvements. Eventually, after 23 days of ventilation support, the patient could be successfully extubated. When the enteral nutrition was re-established and the underlying disease reached the stage of satisfactory improvement she was discharged to an out-patient setting. Conclusions In juvenile dermatomyositis, rituximab may be a promising agent for treatment of rapidly progressive interstitial lung disease. It may also play a role in the therapy of simultaneous pancreatic dysfunction. In the future, the efficacy of rituximab in the refractory form of the disease should be further investigated.