Aortic involvement in giant cell arteritis.

Giant cell arteritis (GCA) is a primary large-vessel vasculitis predominantly seen in the elderly that preferentially involves the external carotid artery and its branches. However, inflammation of the aorta and its branches occurs in a subset of patients although symptoms of aortic involvement may appear years after the initial diagnosis of GCA. Therefore, aortic involvement has probably been underestimated and its incidence may be more frequent than suspected. Systematic evaluation of patients with imaging techniques such as magnetic resonance imaging angiography (MRA) and positron emission tomography (PET) may reveal that the clinical impact of extracranial involvement by GCA may be more relevant than previously thought. Regarding the histopathology, there are some similarities between chronic periaortitis (including idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms and perianeurysmal retroperitoneal fibrosis), idiopathic aortitis, and GCA, suggesting that all these illnesses probably share common pathological mechanisms. Inflammatory aortitis can arise in different clinical settings been idiopathic aortitis more frequent than expected in surgical specimens of aortic aneurysm surgeries in the general population. In the setting of GCA an early diagnosis of aortic involvement is mandatory in order to perform a treatment capable of avoiding the chronic and acute complications associated with an elevated mortality [1, 2].

Colour-duplex ultrasonography of the temporal and ophthalmic arteries in the diagnosis and follow-up of giant cell arteritis.

OBJECTIVE: To evaluate the diagnostic value of colour-duplex ultrasonography (CDU) of the temporal and ophthalmic arteries in the diagnosis of giant cell arteritis (GCA) and its usefulness in the follow-up of the disease. Furthermore, to examine the relationship between CDU abnormalities in ophthalmic arteries and blindness. METHODS: This is a prospective study of all patients with clinical suspicion of GCA or polymyalgia rheumatica (PMR) seen consecutively at the Internal Medicine Department at Vall d'Hebron University Hospital, Spain, between March 2003 and July 2006. Patients were evaluated with regard to the sensitivity and specificity of the dark halo sign in the temporal artery for the diagnosis of GCA, as well as the sensitivity and specificity of the presence of stenosis in temporal and/or ophthalmic arteries. Additionally, the usefulness of the dark halo sign in the follow-up of GCA was addressed. RESULTS: Forty-seven patients (30 with GCA, 17 with PMR) and 13 controls were included in the study. The sensitivity and specificity for the diagnosis of biopsy-proven GCA were higher for the temporal halo (72% in both cases) than for temporal artery stenosis (41% and 89%, respectively), or for ophthalmic artery stenosis (58% and 89%, respectively). Disappearance of the halo was observed in 50% of patients six months after diagnosis, although all patients were in clinical remission, and laboratory parameters were within normal values. CONCLUSION: CDU of the temporal arteries may be a valid tool in the diagnosis of GCA. However, its role in the follow up of the disease deserves re-evaluation. CDU of the ophthalmic arteries is less useful for CGA diagnosis and no relationship with blindness is suspected.

Proposal to decrease the number of negative temporal artery biopsies.

OBJECTIVE: The percentage of negative temporal artery biopsies (TABs) remains very high in patients with suspected giant cell arteritis (GCA). The aim of our study was to identify the clinical predictors of TAB results to improve the effectiveness of this diagnostic procedure. METHODS: We performed a cross-sectional study of 125 consecutive patients who underwent TAB in the Department of Internal Medicine of a public tertiary hospital, from January 1997 to March 2002. We compared patients with a positive and a negative biopsy result. RESULTS: Forty-six of the 125 biopsies (36.8%) were positive for GCA and 79 (63.2%) negative. Temporal artery (TA) thickening (8.01), decreased pulse (5.58), jaw claudication (4.87), and scalp tenderness (4.29) presented the highest positive likelihood ratios (LR+). Erythrocyte sedimentation rate (ESR) (0.11), headache (0.39), TA thickening (0.42), and decreased pulse (0.48) had the lowest negative likelihood ratios (LR-). Only TA abnormalities [odds ratio (OR) 12.3] and scalp tenderness (OR 6) were independent predictors of biopsy results. By excluding patients with no evidence of TA abnormalities, scalp tenderness, or jaw claudication, only 48% of the 125 biopsies would have been performed; 59 negative biopsies would have been prevented, but six positive biopsies would have been missed. CONCLUSION: Careful assessment of patients with clinically suspected GCA to rule out artery abnormalities, scalp tenderness, and jaw claudication can lead to a dramatic decrease in the percentage of negative biopsies. This observation may contribute to improving the physician's decision-making process when contemplating TAB.

[Primary Sjögren's syndrome: clinical and immunological characteristics of 114 patients]. / Síndrome de Sjögren primario: características clínicas e inmunológicas de 114 pacientes.

BACKGROUND: To analyze the clinical and immunological characteristics of a series of 114 patients with primary Sjögren's syndrome (PSS), and to evaluate the different diagnostic criteria and the association to lymphoproliferative disorders. PATIENTS AND METHOD: We included 114 patients (108 female and 6 male) with a diagnosis of PSS. All patients fulfilled the 1993 European Community criteria for the diagnosis of PSS and 76 patients fulfilled the San Diego Criteria. RESULTS: Mean age was 51 years with a mean follow-up of 7.3 years. The commonest clinical manifestation at onset (70%) was xerostomia/xerophtalmia (sicca syndrome). Extra glandular involvement was articular in 42% of cases, neurologic (35%), respiratory (21%) and hepatic (13%). Eleven patients (9%) developed vasculitis, and three (2%) developed a lympho-proliferative disorder. No statistically significant differences regarding symptoms at onset, frequency of glandular or extra glandular manifestations and severity of disease were observed between the two diagnostic criteria groups. HCV infection was associated with vasculitis (p < 0.001; OR: 20.6; CI 95%, 3.2-129) and lymphoproliferative disorders (p < 0.001). CONCLUSIONS: The clinical evolution of PSS does not vary when using different diagnostic criteria (San Diego and European Community criteria). A subset of patients with vasculitis and lymphoproliferative diseases is found to have an associated HCV infection.

Proptosis y tumoración orbitaria en una mujer de 80 años / Proptosis and orbital tumor in a 80-year old female patient

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Respiratory failure due to muscle weakness in inflammatory myopathies: maintenance therapy with home mechanical ventilation.

Polymyositis and dermatomyositis are idiopathic inflammatory myopathies. Respiratory complications are a common feature, but ventilatory insufficiency is rare in these patients. We describe here three patients diagnosed with inflammatory myopathy (polymyositis) with respiratory failure due to muscle weakness who did not respond to immunosuppressive therapy. Mechanical ventilation at home with nasal or tracheal intermittent positive pressure resulted in improved chronic hypoventilation. This treatment improves the quality of life of patients with inflammatory myopathies and can be lifesaving in some cases.

Transverse myelitis in systemic lupus erythematosus: two cases with magnetic resonance imaging.

Transverse myelitis is one of the most serious neurological complications occurring in the course of systemic lupus erythematosus. We describe two lupus patients, with transverse myelitis, one of whom had associated optic neuritis. In both, magnetic resonance imaging of the spinal cord showed an abnormal signal. In one case a good response to steroid and immunosuppressive therapy was observed; the other case failed to improve despite the therapy applied.

Thrombotic thrombocytopenic purpura preceding systemic lupus erythematosus.

The case of a patient admitted with thrombotic thrombocytopenic purpura nine years after developing systemic lupus erythematosus (SLE) is reported. Thrombotic thrombocytopenic purpura associated with SLE has been described on other occasions, but in most patients the diagnosis of SLE precedes that of thrombotic thrombocytopenic purpura. The unusual sequence and the chronological separation of the two diseases is emphasised.

[Echocardiographic manifestations in patients with hypereosinophilia]. / Manifestaciones ecocardiográficas en pacientes con hipereosinofilia.

In order to assess the degree and type of cardiac involvement in patients with sustained hypereosinophilia, we studied by two-dimensional, M-mode echocardiography and Doppler (4 cases) 20 patients distributed into 2 groups. Group I: 10 patients with the idiopathic hypereosinophilic syndrome. Group II: 10 patients with secondary hypereosinophilia. In group I, 6 patients (60%) had echocardiographic abnormalities consistent with the endomyocardial disease: four apical obliteration of right ventricle, three apical obliteration of left ventricle, three endocardial thickening of the left ventricle, three endocardial thickening of the left ventricle posterior wall, one endocardial thickening of the right ventricle free wall, three subvalvular mitral thickening, three subvalvular tricuspid thickening, two pericardial effusion and two protodiastolic septal notch. These corresponded to 2 cases of endomyocardial fibrosis and restriction, as shown by pathological and hemodynamic study. In only 1 patient from group II echocardiographic abnormalities consistent with right apical occupation and tricuspid subvalvular thickening, with mild regurgitation detected by Doppler, were found. It was concluded that echocardiographic abnormalities are common in patients with idiopathic hypereosinophilic syndrome, even in the absence of clinical features. The development of echocardiographic abnormalities in patients with sustained secondary hypereosinophilia is exceptional and is probably related to duration of eosinophilia. Therefore, we think that echo-Doppler is a fundamental investigation for the diagnosis and follow-up of these patients.