[Primary systemic amyloidosis presenting as polymyalgia rheumatica and giant cell arteritis]. / Forma de presentación de una amiloidosis primaria como polimialgia reumática y arteritis de células gigantes.

Primary systemic amyloidosis or AL-amyloidosis is an uncommon disease characterized by the accumulation in vital organs of a fibrillar protein consisting of monoclonal light chains. It is a plasma-cell dyscrasia related to multiple myeloma where clonal plasma cells in the bone marrow produce immunoglobulins that are amyloidogenic. A monoclonal component is present in the serum or urine of 90% of patients. The presentation of most patients with AL amyloidosis is usually related to congestive heart failure, nephrotic syndrome o peripheral neuropathy, but there are unusual features suggesting giant cell arteritis (GCA) and polymyalgia rheumatic (PMR). Although in the majority of AL cases the plasma cells clone is small, the assumption is that the outcome of the disease is uniformly fatal (median survival 12-15 months) and treatment is analogous to those used in malignant proliferative disease. We describe a patient with AL amyloidosis who presented with manifestations of GCA and PMR, and we review the main characteristics of primary amyloidosis.

[Efficacy of quinagolide in the treatment of a patient with hypophyseal resistance to thyroid hormones]. / Eficacia de la quinagolida en el tratamiento de un paciente con resistencia hipofisaria a hormonas tiroideas.

The pituitary resistance to thyroid hormones (PRTH) is not very frequent and well-known entity, their treatment it continues being topic of controversy. In this work we have evaluated the quinagolida effectiveness in the treatment of it unites patient with (PRTH). The relationship among thyroid stimulating hormone (TSH) and free triiodothyronine (FT3) it was used as marker of the thyroid resistance and of the response to the treatment. The concentrations of TSH and FT3 were normalized after adding quinagolida to methimazole. These results suggest that the quinagolida could be an useful drug in the treatment of this pathology, next to the classic treatments.

Eficacia de la quinagolida en el tratamiento de un paciente con resistencia hipofisaria a hormonas tiroideas / Efficacy of quinagolide in a patient with pituitary resistance of thyroid hormone

La resistencia hipofisaria a hormonas tiroideas (RHHT) es una entidad poco frecuente y conocida, su tratamiento sigue siendo tema de controversia. En este trabajo hemos evaluado la eficacia de quinagolida en el tratamiento de una paciente con RHHT. La relación entre tirotropina (TSH) y triyo-dotironina libre (FT3) fue utilizada como marcador de la resistencia tiroidea y de la respuesta al tratamiento. Las concentraciones de TSH y FT3 se normalizaron tras añadir quinagolida al tratamiento antitiroideo clásico con metimazol. Estos resultados sugieren que la quinagolida podría ser un fármaco útil en el tratamiento de esta patología, junto a los tratamientos clásicos. (AU)

Forma de presentación de una amiloidosis primaria como polimialgia reumática y arteritis de células gigantes / Primary systemic amyloidosis presenting as polymialgia rheumatica and giant cell arteritis

La amiloidosis primaria o amiloidosis AL es una discrasia de células plasmáticas difícil de diferenciar del mieloma múltiple, que se caracteriza por el depósito de una proteína fibrilar de cadenas ligeras monoclonales en tejidos y órganos. Es una enfermedad poco frecuente, en la que un clon de células plasmáticas en la médula ósea produce inmunoglobulinas amiloidogénicas. En el 90 por ciento de los casos se detecta por inmunoelectroforesis inmunoglobulinas monoclonales en sangre u orina. Habitualmente se manifiesta como insuficiencia cardiaca, síndrome nefrótico o neuropatía periférica, pero excepcionalmente pueden existir síntomas sugestivos de arteritis de células gigantes (ACG) o polimialgia reumática (PMR). Aunque el número de clones de células plasmáticas en la médula ósea suele ser pequeño, la actitud terapeútica es la de una enfermedad proliferativa maligna, siendo la supervivencia media de 12 a 15 meses.Presentamos un paciente cuya primera manifestación de amiloidosis primaria fueron síntomas de ACG y PMR, y revisamos las principales características de esta enfermedad. (AU)

A randomized cross-over study comparing cabergoline and quinagolide in the treatment of hyperprolactinemic patients.

Quinagolide (QUI) and cabergoline (CAB) are dopamine agonists recently introduced for the treatment of hyperprolactinemia. In the present study, these drugs have been compared in terms of effectiveness and tolerability. Twenty patients (18 females and 2 males) with hyperprolactinemia (8 with microprolactinomas, 6 with idiopathic hyperprolactinemia and 6 with empty sella turcica syndrome) were treated with oral QUI (75 microg once daily) and CAB (0,5 mg twice weekly), in a randomized cross-over trial with placebo between both drugs. Each drug was administered for 12 weeks, separated by other 12 weeks with placebo. PRL levels decreased with both drugs at 2 or 4 weeks of starting the treatment, without differences between both drugs at weeks 4, 8 and 12. At week 12, normal PRL levels (<20 ng/ml) were attained in 90% patients with CAB and only in 75% patients with QUI (p<0.05). After discontinuation of treatment, significant increase in serum PRL was higher after QUI withdrawal than after CAB. Clinical efficacy of both treatments was similar in terms of improvement amenorrhea, oligomenorrhea, galactorrhea, and impotence. All patients completed both cycles of treatment, and the most frequent side-effects were nausea, headache and dizziness, without significant differences between CAB (30%) and QUI (55%). Our study indicates that, at the doses employed here, CAB showed a high percentage of patients with normal PRL at the end of treatment and long-lasting efficacy in the levels of PRL. Clinical response and side-effects were similar in both drugs.

[Levothyroxine treatment of the solitary thyroid nodule]. / Tratamiento con levotiroxina del nódulo tiroideo solitario.

The aim of this study was to review all published randomized clinical trials evaluating the efficacy of suppressive therapy with levothyroxine for solitary thyroid nodules. No significant differences were observed in reducing the volume of benign solitary cold thyroid nodules between the placebo and levothyroxine groups.

[Monoclonal component in visceral leishmaniasis: a rare association that can lead to misdiagnosis]. / Componente monoclonal en leishmaniasis visceral: rara asociación que puede conducir a un falso diagnóstico.

The detection of monoclonal components is exceptional in patients with visceral leishmaniasis (VL). The cases are here reported of two patients with VL in a non-endemic area and monoclonal components which posed problems for the differential diagnosis with other entities associated with the presence of paraproteins. The predominant clinical manifestations in both cases were general symptoms, fever and severe spleen enlargement. One patient had a monoclonal triple band in urine and the other several oligoclonal bands in serum. In the initial bone marrow aspiration smear no parasites were observed in any of the two cases but a remarkable plasmacytosis in one of them. The presence of increased serum titers of anti-Leishmania antibodies was the first demonstrative finding of VL. The diagnosis was confirmed with positive culture of Leishmania and therapy with pentavalent antimonials was successful in both cases. The different diagnostic possibilities are discussed and the possibility of VL is emphasized even in non-endemic areas when monoclonal components in serum or urine specimens are found and consistent clinical findings are present.

Association of Helicobacter pylori infection with cardiovascular and cerebrovascular disease in diabetic patients.

OBJECTIVE: Infection by Helicobacter pylori has been epidemiologically linked to some extradigestive conditions, including ischemic heart disease. Diabetic patients are an at-risk population for cardiovascular and thrombo-occlusive cerebral disease. The aim of the study was to examine a possible relationship between H. pylori infection and cardiovascular or cerebrovascular disease in diabetic patients. RESEARCH DESIGN AND METHODS: This was a cross-sectional case-control study with 127 diabetic patients (both IDDM and NIDDM). Special emphasis was placed on the detection of clinical macro- and microvascular complications, cardiovascular risk factors, acute phase reactants, and serological markers of increased cardiovascular disease risk. H. pylori infection was assessed through the determination of specific Ig-G titers, measured by a commercial enzyme-linked immunosorbent assay. RESULTS: Coronary heart disease was more prevalent in diabetic patients with than without H. pylori (odds ratio [OR] 4.07; 95% CI 1.21-13.6; P < 0.05). A history of thrombo-occlusive cerebral disease was also more frequent in H. pylori-positive diabetic patients (OR 4.8; 95% CI 1.24-18.51; P < 0.05). Other complications such as peripheral arteriopathy, advanced nephropathy, neuropathy, or retinopathy were no differently distributed according to serological status. Alterations in the levels of the following acute-phase reactants and blood chemistry determinations were significantly more profound in H. pylori-positive diabetic patients: high fibrinogen (P < 0.05), high erythrocyte sedimentation rate (P < 0.001), high triglycerides (P < 0.001), and low HDL cholesterol (P < 0.001). There values were also more deeply altered in H. pylori-positive diabetic patients with a history of coronary heart disease, thrombo-occlusive cerebral disease, or both, when compared with H. pylori-positive diabetic patients without those complications. CONCLUSIONS: Our data indicate a possible association of H. pylori infection and the development of coronary heart disease, thrombo-occlusive cerebral disease, or both, in diabetic patients. The importance of this link is highlighted by the possibility of an effective intervention against H. pylori infection.