RESUMEN
Rosai-Dorfman disease is a rare non-Langerhans cell histiocytosis, mainly involving cervical nodes. We present the case of a patient with a pulmonary form of Rosai-Dorfman disease without peripheral or intra-thoracic lymph nodes, characterized by the presence of pulmonary nodules and cysts associated with bilateral pleural effusions.
Asunto(s)
Quistes/etiología , Histiocitosis Sinusal/complicaciones , Nódulos Pulmonares Múltiples/etiología , Derrame Pleural/etiología , Corticoesteroides/uso terapéutico , Anciano , Biopsia , Quistes/diagnóstico , Quistes/tratamiento farmacológico , Quimioterapia Combinada , Histiocitosis Sinusal/diagnóstico , Histiocitosis Sinusal/tratamiento farmacológico , Humanos , Inmunohistoquímica , Inmunosupresores/uso terapéutico , Masculino , Nódulos Pulmonares Múltiples/diagnóstico , Nódulos Pulmonares Múltiples/tratamiento farmacológico , Derrame Pleural/diagnóstico , Derrame Pleural/tratamiento farmacológico , Valor Predictivo de las Pruebas , Tomografía Computarizada por Rayos X , Resultado del TratamientoRESUMEN
We report a case of a 76-year-old woman with isolated unilateral Raynaud phenomenon revealing giant-cell arteritis with diffuse arterial lesions and bilateral renal artery stenosis. Doppler ultrasonography showed bilateral stenosis of the subclavian and axillary arteries. Angio-CT PET enlightened diffuse arterial lesions, mainly involving the aorta and the brachial and femoral arteries as well as bilateral renal ostial stenosis with right kidney ischemia. Diagnosis of giant-cell arteritis was made on the temporal artery biopsy. Corticosteroid therapy led to rapid clinical and radiological improvement. Clinical manifestations of giant-cell arteritis may be atypical. Diffuse arterial disease may exist in the absence of cephalic symptoms or significant inflammatory biological features. Ostial renal artery stenosis may induce potentially threatening renal ischemia.