Reynolds syndrome: a rare rheumatologic disease that internists should have in mind. case report

ABSTRACT Introduction: Reynolds syndrome (RS) is an autoimmune disorder characterized by overlapping primary biliary cirrhosis (PBC) and limited cutaneous systemic sclerosis (lcSSc). Some published cases do not report pulmonary arterial hypertension (PAH), and diagnoses are usually based on clinical, immunological and histological findings, mainly focused on dermatologic features, on those associated with Sjogren's syndrome, or on an interesting presentation of malignant thymoma; only one case of reported PAH was found, but it was an image report. Case Presentation: This paper reports the case of a 75-year-old woman who presented with some of the features mentioned above, severe PAH, dyspnea for one month and two weeks of purulent expectoration, as well as generalized pruritus, nasal telangiectasias, Raynaud phenomenon, sclerodactyly, and high levels of alkaline phosphatase and transaminases. Pulmonary arterial hypertension was documented through a transthoracic echocardiogram, and inmunoflorecence reported mitochondrial and centromere patterns of antinuclear-antibodies. Consequently, RS was diagnosed and phosphodiesterase type-5 inhibitors were started for PAH treatment resulting in the improvement of dyspnea. Conclusion: Dyspnea could be caused by many conditions, but in the presence of clinical and physical findings, it suggests an autoimmune disorder. Scleroderma should be considered and, additionally, PAH should be investigated since it is present in up to 10% of patients, conferring a worse prognosis. Internists should keep in mind that these disorders may be associated with other autoimmune diseases.

Síndrome de Reynolds en Hospital Carlos Andrade Marín, serie de casos y revisión / Reynolds Syndrome in Carlos Andrade Marin Hospital: a series of cases and review

Introducción: El Síndrome de Reynolds es la asociación de esclerosis sistémica (SSC) con cirrosis biliar primaria (CBP). Descrito en 1934 por Milbradt y Reynolds en 1976 describió seis casos. Caso: Presentamos tres casos de mujeres atendidas en el Servicio de Reumatologìa del Hospital Carlos Andrade Marín, con patología hepática autoinmune y signos de esclerosis sistémica. El diagnóstico fue confirmado mediante biopsia hepática en dos de ellas y la prueba de fibromax en la restante. Discusión: Reconocer el Síndrome de Reynolds permite el diagnóstico temprano de cirrosis biliar primaria en pacientes con esclerosis sistémica y sospechar esta entidad en quienes padecen enfermedad hepática colestásica autoinmune que, muchas veces, coexisten en forma silenciosa. El diagnóstico oportuno permite intervenciones terapéuticas precoces que podrían mejorar el pronóstico de esta asociación.

Introduction: The Reynolds' syndrome is the combination of systemic sclerosis (SSC) and primary biliary cirrhosis (PBC). It was first described in 1934 by Milbradt. Reynolds in 1976 presented six cases in two of them and fibromax-test in another. Case report: We present three cases of three women treated in the Rheumatology department at Carlos Andrade Marin hosppital who had autoinmune liver disease confirmed by biopsy. Discusion: Recognizing Reynolds' Syndrome allow us to make earlier diagnosis. This autoimmune cholestatic liver disease often remains silent, so that their identification is a valuable diagnostic tool leading to therapeutic interventions.

Therapeutic effect of integrated traditional Chinese and western medicine therapy on reynolds syndrome / 中国基层医药

Objective To explore therapeutic effect of integrated traditional Chinese and western medicine therapy on reynolds syndrome,and to provide effective and reliable operation method for treatment of the disease. Methods 104 cases with reynolds syndrome were randomly divided into observation group (59 cases)and control group (45 cases)by random number table method.The observation group was treated with aspirin,nifedipine combined with traditional Chinese medicine dialectical therapy,the control group was treated with aspirin and nifedipine. The cold water test ,fist test results and hemorheology indexes of the two groups were measured before and after a period of treatment.The therapeutic effects were evaluated after one course.Results After treatment,the fist test, cold water test positive rates of observation group were 32.2%,23.7%,which were lower than 75.6%,57.8% of the control group (χ2 =19.199,12.504,all P 0.05 ). Conclusion In the treatment of reynolds syndrome,the integrated traditional Chinese and western medicine therapy has better curative effect and low drug adverse reaction rate,with good clinical application value.