[Primary antiphospholipid syndrome: characteristics and course of a series of 17 cases]. / Síndrome antifosfolipídico primario: características y evolución de una serie de 17 casos.

BACKGROUND: Primary antiphospholipid syndrome (APS) is a thrombophilic disorder which is not associated with connective tissue diseases or with other diseases. METHOD: Retrospective review of patients diagnosed of primary APS in an Internal Medicine service between January 1996 and December 2000. RESULTS: We identified 17 patients with primary APS. The most common presenting manifestations were deep vein thrombosis (7 cases) and ischemic stroke (3 cases). Eleven patients (65%) had vein thrombosis, 4 (24%) arterial thrombosis and no patient had both types of thrombosis. The 15 patients with thrombosis received treatment with indefinite oral anticoagulation (INR between 2.5 and 3.5). There was no recurrence of thrombosis or major hemorrhagic complications after a median follow-up time of 36 months (range 10-52 months). CONCLUSIONS: The most common presenting manifestation of primary APS is deep vein thrombosis. Venous thrombosis is more frequent than arterial thrombosis. The occurrence of both types of thrombosis seems to be exceptional. Long-term oral anticoagulation is an effective and safe treatment to prevent the recurrence of thrombosis in patients with primary APS.

[Antineutrophil cytoplasmic antibodies (ANCA): clinico-pathologic correlations iin a series of 82 cases]. / Anticuerpos frente al citoplasma de los neutrófilos (ANCA): correlaciones clínico-patológicas de una serie de 82 casos.

OBJECTIVE: Antineutrophil cytoplasmic antibodies (ANCA) have been described in a great variety of diseases in addition to the ANCA-associated systemic vasculitis (Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS)) and the pauci-immune rapidly progressive glomerulonephritis (RPGN). The objective of this work is to describe the clinical-pathological diagnosis of a series of patients with ANCA. MATERIAL AND METHODS: Retrospective review of patients with positive ANCA by indirect immunofluorescence in our hospital between January 1997 and December 1998. RESULTS: We identify 82 patients with ANCA. Six cases (7.3%) showed a C-ANCA pattern, 19 (23.2%) a P-ANCA pattern and 57 (69.5%) an atypical pattern. Eight patients (9.7%) had an ANCA-associated systemic vasculitis (5 WG, 2 MPA and 1 SCS). Four patients (4.9%) had a pauci-immune RPGN without systemic vasculitis. One only case (1.2%) in our series presented a C-ANCA pattern without evidence of an ANCA-associated systemic vasculitis or a pauci-immune RPGN. CONCLUSIONS: The atypical pattern is the most frequent in patients with ANCA. A minority of patients with ANCA have an ANCA-associated systemic vasculitis (WG, MPA or SCS) or a pauci-immune RPGN. The occurrence of a C-ANCA pattern is exceptional in patients without an ANCA-associated systemic vasculitis or a pauci-immune RPGN.

[The current clinical spectrum of pulmonary thromboembolism]. / El espectro clínico actual del tromboembolismo pulmonar.

BACKGROUND. Mortality in pulmonary thromboembolism (PTE) decreases considerable when it is diagnosed early. The suspicion based on clinical and complementary data is essential for an early diagnosis. METHODS. Retrospective review of the clinical features in patients diagnosed of PTE in an Internal Medicine department from January 1993 to December 1999. RESULTS. A total of 117 patients with PTE were identified. The median age was 68.8 years. Sixty-six patients (56.4%) had one or more risk factors for PTE. The most common risk factor was immobilization (37.6%). Dyspnea was the most common symptom (74.4%) and tachypnea the most common sign (66.7%). Fever/low grade fever and leukocytosis were present in 16.2% and 31.6% of patients, respectively. Respiratory failure, alkalosis and hypocapnia were present in 44.4%, 38.5% and 47% of patients, respectively. An alveolar-arterial oxygen gradient > 20 mmHg was demonstrated in 96.6% of patients. Chest radiographs and electrocardiograms were normal in 52.1% and 23.9% of patients, respectively. A vein echo-duplex of the lower limbs demonstrated deep vein thrombosis (DVT) in 52.1% of patients. The hospital mortality rate was 6.8%. CONCLUSIONS. PTE still affects older patients mainly and frequently known risk factors are not detected. The presence of fever/low grade fever and/or leukocytosis does not rule out PTE. Both chest radiographs and electrocardiograms may be normal. Not demonstrating DVT in the lower limbs by the vein echo-duplex does not rule out PTE. The hospital mortality rate has not decreased considerably in the last few years.

[Differences between tuberculous spondylitis and brucellar spondylitis]. / Diferencias entre la espondilitis tuberculosa y la espondilitis brucelar.

OBJECTIVE: To identify potential differences in the clinical and laboratory characteristics between tuberculous spondylitis (TS) and brucellar spondylitis (BS). PATIENTS AND METHODS: Retrospective study of patients with TS and BS diagnosed in our hospital between january 1992 and december 1998. RESULTS: TS was diagnosed in 17 patients and BS in 10 patients. In our series, a higher delay in the diagnosis (27.9 +/- 24.6 vs. 16 +/- 5.6 weeks, p = 0.02) was found in TS. There was a higher frequency, but without stadistic significance, of immunosuppression, one or several paravertebral or epidural abscesses, spinal cord compression, anemia and an elevated erythrocyte sedimentation rate in TS, and a higher frequency of fever/febricule and residual vertebral pain in BS. Lumbar location was the most frequent in both groups (58.8% in TS and 70% in BS). CONCLUSIONS: It is possible that there were some differences in the clinical and laboratory characteristics between TS and BS which may be an aid in the differential diagnosis of both entities and orient the empirical treatment in these cases without a definitive microbiological diagnosis or while awaiting the diagnostic confirmation.

[Giant-cell arteritis: a descriptive study in southwestern Spain]. / Arteritis de células gigantes: un estudio descriptivo en el suroeste de España.

OBJECTIVE: To study the clinical and laboratory features of a series of patients with giant cell arteritis (GCA) or temporal arteritis in south-western Spain (Extremadura). PATIENTS AND METHODS: Retrospective study of 25 patients with GCA diagnosed by temporal artery biopsy between 1990 and 1998. RESULTS: Nine patients were males and 16 (64%) females. Sixteen cases (64%) presented polymyalgia rheumatica (PMR). Other clinical findings were: fever/febricula (64%), constitutional syndrome (64%), new headache (96%), visual symptoms (48%), jaw claudication (17%) and abnormal temporal arteries (17%). All patients had an ESR of more than 50 mm/hour and a raised C-reactive protein. Thirteen patients (52%) had anemia (hemoglobin level < 12 g/dl). Eleven cases (44%) presented a platelet count higher than 400,000/mm3. Four patients (16%) had an elevated AST and/or ALT levels and 8 patients (32%) had an elevated GGT and/or alkaline phosphatase levels. In patients with PMR, there was a higher frequency of constitutional syndrome (81 vs 33%, p = 0.02). In females, there was a higher frequency of anemia (75 vs 11%, p < 0.01), platelet count higher than 400,000/mm3 (75 vs 0%, p < 0.01) and elevated AST and/or ALT (25 vs 0%, p < 0.01) and elevated GGT and/or alkaline phosphatase (50 vs 0%, p < 0.01) levels. CONCLUSIONS: The clinical and laboratory features of GCA in our series of patients in south-western Spain are similar to that described in other spanish populations, with the exception of a slightly higher frequency of PMR and a lower frequency of jaw claudication and abnormal temporal arteries. In our study, the clinical picture of GCA was more severe in patients with PMR and in females.

[Osteopoikilosis: report of 3 cases and review of the literature]. / Osteopoiquilosis: presentación de tres casos y revisión de la literatura.

Osteopoikilosis, osteopathia condensans disseminata, is a rare hereditary autosomal dominant sclerosing bone dysplasia, more common in males. The diagnosis is usually made incidentally from radiographs which show multiple, small, well-defined, variably shaped and widely distributed (over the skeleton) sclerotic areas. The involvement is symmetrical, and the predilected locations are the phalanges of the hand, carpal bones, metacarpals, foot phalanges, metatarsals, tarsal bones, ilium, femur, radio and sacrum. It must be distinguished from melorheostosis, osteopathia striata and fundamentally from osteoblastic bone metastases, on the basis of the clinical, radiological (roentgenographs, computed tomography and magnetic resonance) and radionuclide scanning characteristics. Histologically, there are focal condensations of compact lamellar bone within the spongiosa. We report three cases of osteopoikilosis and review the literature. Two cases didn't have affectation in phalanges of the hand, which had not been previously reported, to our knowledge.

[Rheumatic polymyalgia]. / Polimialgia reumática.

Polymyalgia rheumatica (PMR) is a common clinical syndrome that is characterized by pain and stiffness in neck, shoulder girdle and pelvic girdle. The aetiology is unknown. However, recent studies have documented an association with HLA antigens and infectious agents. It occurs mostly after the age of 50 years and is often accompanied by systemic features such as fever, asthenia, hyporexia and weight loss. An erythrocyte sedimentation rate (ESR) of at least 40 mm/hour has been considered diagnostic criterion. Nevertheless, a normal ESR accounted for up to 20% of cases of PMR. A dramatic and prompt response to corticosteroid treatment is characteristic. Giant cell arteritis/temporal arteritis (GCA) has been found in 0-80% of cases of PMR. Temporal biopsy could initially be deferred in patients with PMR younger than 70 years with no cranial symptoms, in which the risk of GCA is very low.

Recurrence of adrenal aldosterone-producing adenoma.

Conn's syndrome (adrenal aldosterone-producing adenoma) and bilateral adrenal hyperplasia are the most common causes of primary aldosteronism. The treatment of choice for patients with aldosterone-producing adenoma is unilateral total adrenalectomy. Recurrence after adequate surgery is exceptional. We present a patient with recurrence of an aldosterone-producing adenoma in the right adrenal gland 9 years after adenomectomy of a aldosterone-producing adenoma in the same adrenal gland. We conclude that adenomectomy is not an adequate therapy for patients with adrenal aldosterone-producing adenoma.

[Symptomatic sinus bradycardia associated with donepezil]. / Bradicardia sinusal sintomática asociada a donepecilo.

INTRODUCTION: Donepezil is a drug which is being used more and more widely in mild-moderate Alzheimer's disease. In general, it is well tolerated and the side-effects are basically cholinergic-dependent. Symptomatic disorders of cardiac rhythm associated with the use of donepezil are extremely unusual. CLINICAL CASE: We describe the case of an 81 year old patient with hypertensive cardiopathy, who developed sinus bradycardia, fainting and left cardiac failure three weeks after starting treatment with donepezil. When donepezil was stopped the sinus bradycardia disappeared, a 24 hour electrocardiographic holter showed no signs of sinus node disease and no episodes of this type occurred during the following six months. CONCLUSION: Symptomatic sinus bradycardia is a possible adverse effect of treatment with donepezil in Alzheimer's disease.