CHEST Guidelines-Juvenile-Dermatomyositis-and-Acute-Respiratory-Fai

Juvenile-Dermatomyositis-and-Acute-Respiratory-Fai

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This case study presents a 17-year-old Taiwanese girl diagnosed with juvenile dermatomyositis (JDM) and rapid progression to acute respiratory failure. Initially, she experienced symptoms such as syncope, weakness, polyarthritis, and a characteristic heliotrope rash. Investigations confirmed JDM with elevated creatine phosphokinase levels and diffuse myositis. Despite treatment with oral steroids and methotrexate, her condition deteriorated, leading to treatment escalation with pulse methylprednisolone and cyclophosphamide.<br /><br />The patient later developed acute symptoms, including fever, cough, and worsening hypoxemia, compounded by Staphylococcus aureus bacteremia and Pneumocystis jiroveci infection. Although treated with antibiotics, she required full-time ventilatory support due to hypoxemia and underwent a tracheostomy. Imaging revealed interstitial lung disease (ILD) and her high oxygen needs exceeded her neuromuscular status, prompting consideration for lung transplantation.<br /><br />Her positivity for anti-MDA5 antibodies, commonly associated with rapid progression to ILD, led to a decision for a lung transplant, the only life-saving intervention available. Two months post-diagnosis, extensive treatment including immunosuppressants and venovenous extracorporeal membrane oxygenation facilitated her surviving until a bilateral lung transplant could be performed. Pathology revealed bilateral acute onset end-stage diffuse fibrosing lung disease.<br /><br />Post-transplant, she showed improvement and normal activity tolerance while remaining on immunosuppressive therapy with tacrolimus, mycophenolate mofetil, and prednisone. Clinical recommendations emphasize the need for early referral for lung transplantation for patients with anti-MDA5-positive JDM and rapidly progressive ILD due to associated high morbidity and mortality rates. Despite its rarity, close monitoring for ILD in JDM patients with anti-MDA5 positivity is critical.

Keywords

juvenile dermatomyositis

acute respiratory failure

heliotrope rash

interstitial lung disease

lung transplantation

anti-MDA5 antibodies

Staphylococcus aureus

Pneumocystis jiroveci

immunosuppressive therapy

tacrolimus