Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group.

OBJECTIVES: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death. METHODS: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group. RESULTS: Twenty-one referral centers participated in the registry. By April 2014, 1374 patients with SSc had been enrolled, and 595 of whom (43%) had ILD: 316 (53%) with limited cutaneous SSc (lcSSc), 240 (40%) with diffuse cutaneous SSc (dcSSc), and 39 (7%) with SSc sine scleroderma (ssSSc). ILD in the lcSSc and the ssSSc subsets tended to develop later, and showed a less impaired forced vital capacity (FVC) and a ground glass pattern on high-resolution computed tomography (HRCT) less frequently, compared with the dcSSc subset. Factors related to an FVC < 70% of predicted in the multivariate analysis were: dcSSc, positivity to anti-topoisomerase I antibodies, a ground glass pattern on HCRT, an active nailfold capillaroscopy pattern, lower DLco, older age at symptoms onset, and longer time between symptoms onset and ILD diagnosis. Finally, SSc-associated mortality and ILD-related mortality were highest in dcSSc patients, whereas that related to pulmonary arterial hypertension was highest in those with lcSSc-associated ILD. CONCLUSIONS: Our study indicates that ILD constitutes a remarkable complication of SSc with significant morbidity and mortality, which should be borne in mind in all three subgroups (lcSSc, dcSSc, and ssSSc).

Systemic sclerosis sine scleroderma and limited cutaneous systemic sclerosis: similarities and differences.

OBJECTIVES: To compare a cohort of patients with systemic sclerosis sine scleroderma (ssSSc) vs. patients with limited cutaneous systemic sclerosis (lcSSc). METHODS: Forty-five patients with ssSSc and 186 patients with lcSSc were investigated. Demographic, clinical and immunologic features and survival were compared. RESULTS: There were no significant differences between ssSSc and lcSSc in gender, age at onset and interval between onset and diagnosis. ssSSc patients fulfilled the ACR criteria for SSc less than lcSSc patients (13%/77%, p<0.0001). There were no significant differences in articular involvement, myopathy, tendon friction rubs and gastrointestinal, pulmonary, cardiac and renal involvements. There was a trend to higher prevalence of pulmonary arterial hypertension (PAH) in ssSSc patients (29%/19%) but not reach significant difference. The prevalence of antinuclear and anticentromere antibodies and slow capilaroscopic pattern was similar. Sicca syndrome (13%/30%; p=0.024), digital ulcers (16%/50%; p<0.0001), calcinosis (11%/26%; p=0.047) and acroosteolysis (0% /10%; p=0.028) were more frequently in lcSSc. Survival at 5, 10, and 15 yr was not different in ssSSc and lcSSc patients (100%/98%, 100%/98%, and 92%/89%, respectively). CONCLUSIONS: ssSSc and lcSSc patients share demographic, clinical and immunologic features. Survival is also similar in both groups. Differences are mainly due to peripheral vascular manifestations. However, despite great similarities, we believe that ssSSc patients should be considered as a different subset in order to avoid misdiagnosis. ssSSc patients should be truly differentiated from early SSc using sensitive and specific studies looking for any asymptomatic organ involvement.

Intravenous cyclophosphamide pulse therapy in the treatment of systemic sclerosis-related interstitial lung disease: a long term study.

OBJECTIVE: Interstitial lung disease (ILD) frequently complicates systemic sclerosis (SSc). Cyclophosphamide (CYC) is a promising immunosuppressive therapy for SSc-related ILD. Our objective was to investigate the effectiveness of an intravenous CYC (iv CYC) pulse regime in SSc-related ILD during treatment and thereafter. METHODS: In a prospective observational study ten consecutive patients with SSc-related ILD were treated with iv CYC in a pulse regime lasting from 6 to 24 months. Clinical status, pulmonary functional testing (PFT) and high resolution computed tomography (HRCT) of the chest were evaluated at enrolment and 6, 12 and 24 months thereafter. After treatment withdrawal, patients were followed up every 6 months with PFT and chest HRCT to monitor lung disease. RESULTS: Clinical improvement was apparent in 8 out of 10 patients. The median values of forced vital capacity (FVC), forced expiratory volume in the first second (FEV1) and diffusion lung capacity for carbon monoxide (DLCO) as well as ground-glass pattern on HRCT did not change significantly after 6, 12 and 24 months of therapy. The follow-up continued in 8 out of 10 patients after treatment withdrawal for a median of 26.5 months (range: 12-48 months). The final median FVC was 54.5% of predicted value (interquartile range, IQR= 31.6%-94%). Only one patient suffered a FVC deterioration greater than 10%, even though less than 160 ml. The final median DLCO was 68% of predicted value (IQR=38.3-83.6%). Only 2 patients who developed pulmonary arterial hypertension deteriorated their DLCO values of more than 15%. CONCLUSIONS: An iv CYC pulse regimen over 24 months may stabilize pulmonary activity in patients with SSc-related ILD during the course of treatment and for a median of 26.5 months thereafter.

[Analysis of a series of 55 patients with biopsy proven giant cell temporal arteritis]. / Análisis de una serie de 55 pacientes con arteritis de células gigantes confirmada por biopsia.

INTRODUCTION: Our main aim with this study is to establish the epidemiologic and clinical features, treatment response and complications of a group composed of 55 patients with biopsy proven temporal arteritis in a local hospital. MATERIAL AND METHODS: Retrospective study based on clinical records revision of patients diagnosed of giant cell arteritis (GCA) made by temporal artery biopsy between 1989 and 2001. RESULTS: The approximated annual incidence of GCA in our area is 4.1 cases per 100,000 persons over the age of 50. The mean age at diagnostic was 74 years and the 78,2% were women. The most common symptom at diagnostic was headache (81.5%) followed by systemic manifestations (74.1%) and later we found jaw claudication (32,7%), visual impairment (30.2%), isquemic manifestations (17%). Polymyalgia rheumatica was associated to GCA in 49.1% of cases. The temporal artery explorations was abnormal in the 76.9% of patients. The mean initial dose of corticoids was 69 mg prednisone per day, with a half dose reduction time of .5 months. In spite of that, 24,1% of patients relapsed during the first year. The 38% of patients did some complications during the corticosteroid treatment. The ESR was lower 50 mm in 12.7% of patients; it was anaemia in the 37.7% and thrombocytosis in 32,1%. In these last patients we detected a relation between thrombocytosis and specific visual impairment. CONCLUSIONS: The incidence of GCA in our area is low. The results of our series aren't different from others publicated before in clinical manifestations, there is a prevalence of female sex and there is a relation between specific visual impairment and the presence of thrombocytosis.

Análisis de una serie de 55 pacientes con arteritis de células gigantes confirmada por biopsia / Analysis of a serie of 55 patients with biopsy proven giant cell temporal arteritis

Introducción: Descripción de las características epidemiológicas, clínicas, respuesta al tratamiento y complicaciones de una serie de 55 pacientes con arteritis de células gigantes(ACG) confirmada por biopsia en un hospital comarcal. Material y métodos: Estudio retrospectivo de los pacientes diagnosticados de ACG por biopsia de arteria temporal en el periodo comprendido entre 1989 y 2001 en nuestro centro. Resultados: La incidencia anual aproximada calculada en nuestra área sería de 4,1 casos por 100.000 habitantes mayor de 50 años. La edad media al diagnóstico fue de 74 años y un 78,2 por ciento eran mujeres. El síntoma más frecuente al diagnóstico fue la cefalea (81,5 por ciento), seguido de las manifestaciones sistémicas (74,1 por ciento), a más distancia se encontraba la claudicación mandibular (32,7 por ciento), trastornos visuales (30,2 por ciento), manifestaciones isquémicas (17 por ciento). La polimialgia reumática se asoció a ACG en el 49,1 por ciento. La palpación de arteria temporal fue anormal en el 76,9 por ciento de los pacientes. La dosis media de inicio de tratamiento fue de 69 mg prednisona /dia, con una media de tiempo de reducción a mitad de dosis fue de 3,5 meses. Un 24,1 por ciento de los pacientes presentaron un rebrote durante el primer año. Un 38 por ciento de los pacientes presentaron complicaciones durante el tratamiento corticoideo. La VSG fue inferior a 50 mm en el 12,7 por ciento de los pacientes, existía anemia en el 37,7 por ciento y trombocitosis en el 32,1 por ciento. En estos últimos se detectó una tendencia a la relación con presencia de alteraciones visuales específicas. Conclusiones: La incidencia de ACG en nuestra área estaría dentro de las zonas de baja incidencia. Los resultados de nuestra serie no difieren de otras descritas previamente en la literatura en cuanto a las manifestaciones clínicas de los pacientes, existe un claro predominio del sexo femenino y se detecta una tendencia a la relación de alteraciones visuales específicas con presencia de trombocitosis (AU)

Profilaxis primaria y secundaria de la enfermedad tromboembólica venosa en pacientes con enfermedades autoinmunes sistémicas / No disponible

No disponible

Mesangioproliferative glomerulonephritis and antibodies to phospholipids in a patient with acute Q fever: case report.

Glomerulonephritis is a rare complication of Q fever and is usually associated with the chronic form of disease, i.e., infectious endocarditis. We describe a 31-year-old man with a self-limited acute febrile illness due to Coxiella burnetii infection who had acute renal failure secondary to mesangioproliferative glomerulonephritis. Antibodies to phospholipids were detected and suggested the diagnosis of Q fever in this case.

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.

[A cholesterol embolism following coronary angioplasty. A case report]. / Embolismo de colesterol tras angioplastia coronaria. Presentación de un caso.

Cholesterol embolism is a rare but serious complication of heart catheterization. We report a patient in whom cholesterol embolization syndrome developed after coronary angioplasty complicated by an acute myocardial infarction which was treated with streptokinase and heparin. The clinical outcome was satisfactory. Cholesterol embolism occurrence might have been precipitated in this patient by thrombolytic and anticoagulant therapy.

Raynaud's phenomenon and positive antinuclear antibodies in a malignancy.

Both Raynaud's phenomenon and the presence of antinuclear antibodies are uncommon features of malignant disease and the association of both with a malignancy extremely rare. The case is reported of a 78 year old woman who presented with Raynaud's phenomenon and positive antinuclear antibodies related to adenocarcinoma of unknown primary site.

[False positive serological tests for Borrelia burgdorferi in patients with primary antiphospholipid syndrome]. / Serología falsamente positiva frente a Borrelia burgdorferi en pacientes con síndrome antifosfolípido primario.

Serological tests for Borrelia burgdorferi were carried out in a series of 27 patients with primary antiphospholipid syndrome (PAPLS). The results showed a 14% prevalence of false positive Lyme disease serology in this patient population. In patients with PAPLS and false positive serologic tests for Borrelia burgdorferi neurological involvement was significantly more common (p = 0.012, Fisher's exact test) than in patients with PAPLS without that finding. We insist on the possibility that patients with PAPLS may have false positive serological tests for Borrelia burgdorferi as measured by IFI with the resulting diagnostic error. In these cases, confirmation by other techniques such as ELISA or immunoblotting is recommended.