RESUMEN
IgG4-related disease is a heterogeneous group of diseases that can affect a single organ or manifest as a systemic disease. We present the case of a 55-yearold female, admitted for dry cough, fever, progressive dyspnea and respiratory failure. Chest CT showed areas of diffuse ground glass, reticular pattern and alveolar consolidation. She received treatment for community acquired pneumonia and systemic corticosteroids with good response. One month later, she was admitted again due to subglottic stenosis, progression of pulmonary infiltrates, and increased palpebral and submaxillary glands volume. Transbronchial and salivary gland biopsies showed infiltration by IgG4. Increased levels of plasma IgG4 were also detected. Immunosuppressive therapy was given with good response.
Asunto(s)
Enfermedad Relacionada con Inmunoglobulina G4/patología , Enfermedades Pulmonares Intersticiales/patología , Biopsia , Femenino , Humanos , Enfermedad Relacionada con Inmunoglobulina G4/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/inmunología , Persona de Mediana Edad , Neumonía/tratamiento farmacológico , Neumonía/patología , Tomografía Computarizada por Rayos XRESUMEN
Las enfermedades relacionadas con inmunoglobulina G4 (IgG4) son un grupo heterogéneo de cuadros clínicos que pueden afectar un solo órgano o tener compromiso sistémico. Se presenta el caso de una mujer de 55 años, internada por un cuadro de tos seca, fiebre, disnea progresiva e insuficiencia respiratoria. La tomografía de tórax evidenció áreas de vidrio esmerilado difusas, patrón reticular y consolidación alveolar. Recibió tratamiento habitual para neumonía de la comunidad y corticoides sistémicos, presentando buena evolución y otorgándose el alta. Al mes se reinterna por estenosis subglótica, progresión de infiltrados pulmonares y aumento del volumen palpebral y de las glándulas submaxilares. Se realizaron biopsias transbronquial y de glándulas salivales que mostraron infiltración por IgG4. También se detectó aumento de los niveles de IgG4 en plasma. Recibió tratamiento con inmunosupresores evolucionando con buena respuesta.
IgG4-related disease is a heterogeneous group of diseases that can affect a single organ or manifest as a systemic disease. We present the case of a 55-year-old female, admitted for dry cough, fever, progressive dyspnea and respiratory failure. Chest CT showed areas of diffuse ground glass, reticular pattern and alveolar consolidation. She received treatment for community acquired pneumonia and systemic corticosteroids with good response. One month later, she was admitted again due to subglottic stenosis, progression of pulmonary infiltrates, and increased palpebral and submaxillary glands volume. Transbronchial and salivary gland biopsies showed infiltration by IgG4. Increased levels of plasma IgG4 were also detected. Immunosuppressive therapy was given with good response.