ABSTRACT
Interstitial pulmonary fibrosis is the leading cause of secondary pulmonary hypertension in systemic sclerosis, and it occurs in either limited or diffuse cutaneous scleroderma subset. Isolated pulmonary hypertension, without pulmonary disease, occurs primarily in patients with limited cutaneous scleroderma (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia [CREST] variant) although it is an unusual feature in this subset, with a worse prognosis in the short term. We present a previously undiagnosed patient with the CREST syndrome, with severe isolated pulmonary hypertension and secondary respiratory failure as major feature of its connective tissue disease. Clinical, prognostic and therapeutical aspects are commented.
Subject(s)
CREST Syndrome/complications , CREST Syndrome/diagnosis , Hypertension, Pulmonary/etiology , Aged , Female , Humans , Severity of Illness IndexABSTRACT
La fibrosis pulmonar intersticial es la causa más frecuente de hipertensión pulmonar secundaria en la esclerosis sistémica, tanto en el subgrupo de escleroderma cutánea difusa como en el de escleroderma cutánea limitada. La hipertensión pulmonar aislada, no asociada a enfermedad pulmonar, aunque ocurre primariamente en pacientes con escleroderma cutánea limitada y su variante el síndrome CREST (calcinosis, fenómeno de Raynaud, dismotilidad esofágica, esclerodactilia y telangiectasias) constituye un hallazgo infrecuente en este subgrupo, implicando un mal pronóstico a corto plazo. Comunicamos el caso de una paciente con síndrome CREST, no diagnosticada previamente, con hipertensión pulmonar aislada severa e insuficiencia respiratoria secundaria como manifestación principal de su conectivopatía. Se comentan aspectos relacionados con la clínica, pronóstico y manejo terapéutico de estos pacientes (AU)
Subject(s)
Aged , Female , Humans , Hypertension, Pulmonary , Severity of Illness Index , CREST Syndrome , Hypertension, Pulmonary/etiology , CREST Syndrome/complications , CREST Syndrome/diagnosisABSTRACT
Lymphocytic colitis is a rare clinicopathologic syndrome, characterized by chronic watery diarrhea, diffuse inflammatory changes in the colonic mucous in spite of normal findings on colonoscopy and marked intraepithelial lymphocytic infiltration on biopsy. Although the physiological mechanism of diarrhea is not clear, patients do not usually present hydroelectrolytic alterations and the results of routine laboratory investigations are usually normal. The association between lymphocytic colitis and thyroid disease, possibly autoimmune, in the form of hypo- or hyperthyroidism is relatively common. We report a 61-year-old woman with a history of multinodular toxic goiter, whose previously uninvestigated chronic diarrhea became more acute and led to the diagnosis of lymphocytic colitis. Results of laboratory investigations revealed only a significant hypokalemia with an associated nonfunctioning bilateral adrenal incidentaloma. The patient evolved well when treated with sulfasalazine. Hypokalemia as a complication of lymphocytic colitis and an association between lymphocytic colitis and toxic multinodular goiter does not seem to have been previously described.
Subject(s)
Colitis/complications , Graves Disease/complications , Hypokalemia/etiology , Lymphocytes , Acute Disease , Chronic Disease , Colitis/diagnosis , Colitis/drug therapy , Diagnosis, Differential , Diarrhea/diagnosis , Diarrhea/drug therapy , Diarrhea/etiology , Female , Gastrointestinal Agents/therapeutic use , Graves Disease/diagnosis , Graves Disease/drug therapy , Humans , Hypokalemia/diagnosis , Hypokalemia/drug therapy , Middle Aged , Sulfasalazine/therapeutic useABSTRACT
A patient with advanced multiple myeloma (MM) and renal failure presented a severe chronic anemia requiring frequent blood transfusions. Treatment with recombinant human erythropoietin (rHuEPO) led to a rapid improvement of anemia, and further blood transfusions were not required. Pathophysiological studies about the erythropoiesis in patients with MM and trials with rHuEPO in myeloma-associated anemia are commented.
