ABSTRACT
La sarcoidosis es un trastorno crónico, multisistémico y granulomatoso. Femenina de 2 años con múltiples cuadros de vías respiratorias, con esteroides inhalados y sistémicos presentando respuesta parcial, reactantes de fase aguda positivos, anticuerpos negativos, además de PCR de micobacterias atípicas con Mycobacterium tuberculosis negativa. Se diagnostica de sarcoidosis laríngea por histopatología biopsia de laringe con proceso inflamatorio crónico granulomatoso con células gigantes tipo Langhans, y áreas agudizadas y ulceradas con cambios compatibles con proceso fímico. Se inicia tratamiento con gammaglobulina mensual durante 6 meses a dosis de 2g/kg/peso, Valmetrol(R) y metotrexate. Se decidió la inmunomodulación con gammaglobulina y con posterior uso de inmunosupresión a base de metotrexate. Actualmente broncoscopia sin evidencia de granulomas y reactantes de fase aguda negativos
Sarcoidosis is a chronic, multisystemic, granulomatous disorder. Our patient was a 2-year-old girl with multiple airway conditions and a partial response to inhaled and systemic steroids. She was positive for acute phase reactants and negative for antibodies. Polymerase chain reaction revealed atypical Mycobacteria and she was negative for Mycobacterium tuberculosis. Laryngeal sarcoidosis was diagnosed by histopathology in a biopsy of larynx that revealed a chronic granulomatous inflammatory process with Langhans giant cells and acute and ulcerated areas with changes compatible with tuberculosis. Treatment consisted of monthly gammaglobulin for 6 months at doses of 2g/kg body weight, accompanied by Valmetrol(TM) and methotrexate. Immunomodulation with gammaglobulin was prescribed, with subsequent use of methotrexate-based immunosuppression. Currently, bronchoscopy shows no evidence of granulomas and she is negative for acute-phase reactants
Subject(s)
Humans , Female , Child, Preschool , Laryngeal Diseases/diagnosis , Sarcoidosis/diagnosisABSTRACT
Sarcoidosis is a chronic, multisystemic, granulomatous disorder. Our patient was a 2-year-old girl with multiple airway conditions and a partial response to inhaled and systemic steroids. She was positive for acute phase reactants and negative for antibodies. Polymerase chain reaction revealed atypical Mycobacteria and she was negative for Mycobacterium tuberculosis. Laryngeal sarcoidosis was diagnosed by histopathology in a biopsy of larynx that revealed a chronic granulomatous inflammatory process with Langhans giant cells and acute and ulcerated areas with changes compatible with tuberculosis. Treatment consisted of monthly gammaglobulin for 6 months at doses of 2g/kg body weight, accompanied by Valmetrol™ and methotrexate. Immunomodulation with gammaglobulin was prescribed, with subsequent use of methotrexate-based immunosuppression. Currently, bronchoscopy shows no evidence of granulomas and she is negative for acute-phase reactants.
