36º Congresso Brasileiro de Reumatologia

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Title

SYSTEMIC ERITEMATOSUS LUPUS WITH PULMONARY HEMORRHAGE IN ADOLESCENTS: CASE REPORT

Background

Systemic Lupus Erythematosus (SLE) is the prototypic autoimmune disease. It is characterized by multiple autoantibodies associated with multisystem illness. Pulmonary alveolar hemorrhage (PAH) is a relatively rare but potentially catastrophic event ( mortality rate 50%). PAH patients rarely have hemoptysis but may have fever, cough,and dyspnea. Computed tomography (CT) scan reveals pulmonar nodules and ground glass changes in patients who may have patchy infiltrates or normal-appearing chest radiographs.

Case report

12-year-old girl with previous history of atopic dermatitis with prior use of metrotexate and folic acid was diagnosed with systemic lupus erythematosus in August 2017. When he started hemoptotic sputum, productive cough and dyspnea, progressing to severe respiratory failure and need for orotracheal intubation and Intensive Care Unit (UTI). She was hospitalized and stabilized in Emergency Hospital and referred to UTI´ Pediatric presenting improvement after antibiotic therapy and critical care support. She followed with gradual clinical improvement but had persistent productive cough with orange sputum. FAN 1: 320 dotted nuclear patterns and anti-SM reagent (151 U / mL) confirmed SLE. Etiology of the initial respiratory condition (SLE activity or the consequence of previous metrotexate intoxication), was investigate CT scan of the chest revealed peripheral and just pleural pulmonary nodules in the posterior basal segment of the upper lobe and nonspecific interstitial pneumonia bilaterally. Pulmonary biopsy revealed inflammatory process, edema, hemorrhage and emphysematous areas compatible with severe SLE alterations. Indicated combined pulse therapy: methylprednisolone and cyclophosphamide. Patient presented gradual improvement receiving discharge from hospital after 4 months.

Conclusion

Pulmonary hemorrhage in SLE in childhood and adolescence a lot more aggressive disease with catastrophic consequences to the patient. Diagnosis and early therapy may improve prognosis.

Área

Pediatrics

Autores

Catarina Fernandes Pires, Lucas Aguiar Alencar Oliveira, Aparecida Maísa de Carvalho Gomes, Roberta Oriana Assunção Lopes Sousa, Maria do Socorro Teixeira Moreira Almeida, Lorena Rodrigues Madeira Moura Fé, Rafaela Santos Martins