Bronchoalveolar lavage in systemic sclerosis with lung involvement: role and correlations with functional, radiological and scintigraphic parameters

To evaluate the role and the prognostic value of bronchoalveolar lavage (BAL) in scleroderma patients withinterstitial lung disease. We reviewed the records of 79 patients with systemic sclerosis (SSc) who had dyspnea and pulmonary involvement and underwent BAL study. Sixty-two patients were prospectively followed up for 12–36 months and re-evaluated by pulmonary function tests (PFTs). Seventy-nine SSc patients were enrolled (71 F and 8 M), 55 with limited and 24 with a diVuse form; mean age55 +/- 13 years; mean disease duration 55.2 +/- 59 months. All patients were ANA positive, of these 30 were anti-topoisomerase-1 positive (anti-Topo1) and 22 were anti-centromere positive (ACA). Thirty-one patients had alveolitis (39.2%) that was neutrophilic in 12 patients, eosinophilic in 3 and mixed (neutrophilic and eosinophilic) in 16 patients. Compared to patients without alveolitis, those with alveolitis had a significant reduction of carbon monoxide diffusing capacity (DLCO), forced vital capacity (FVC) and more elevated lung high-resolution computed tomography (HRCT) scores. Furthermore, alveolar clearance was signiWcantly accelerated. No differences were found between patients with and without alveolitis regarding disease subsets (diffuse vs limited-SSc); a significant predominance of anti-Topo1 antibodies was foundin the alveolitis group and of ACA antibodies in the non-alveolitiscohort. During the follow-up, (range: 12–36 months) 62patients, 26 with and 36 without alveolitis were re-evaluated with PFTs. In the alveolitis group, 12 patients (46.1%)showed stable lung function parameters and 14 had worsened (53.8%). In this group, 20 patients (77%) received cyclophosphamide (CYC): 11 (55%) worsened (5 of them died of cardio-pulmonary complications) and 9 (45%) remained stable. Six patients could not be treated; of these 3 remained stableand 3 worsened. Among 36 patients with normal BAL, 11 (30.5%) showed stable lung function parameters, 13 improved (36.1%) and 12 worsened (33.3%); in this last group, 2 patients died of extra-pulmonary complications. Six patients, with progression of lung fibrosis, were treated with CYC: 3 of them improved and 3 remained stable. Our study revealed a trend toward a more severe course in the SSc patients with BAL alveolitis; probably the non-significant result is related to the low number of the examined subjects and to the selection criteria. However, BAL remains the only tool to exclude lung infections and, in our experience, a useful instrument to evaluate interstitial lung disease in SSc patients

Headache in Behçet’s disease: case reports and literature review

Objective: To evaluate the prevalence of headache and its different patterns in patients with Behçet’s disease (BD) with and without neurological involvement and to investigate clinical correlations. Methods: Patients fulfilling the International Study Group criteria for Behçet disease (ISGc) were studied. Patients were invited to fill a “headache questionnaire”, which consisted of two sections: the first one included demographic and anamnestic data, family history for both headache and BD, disease duration and clinical manifestations of BD; the second section included items about headache, investigated accordingly to International Headache Society diagnostic criteria (IHS, 2004). Clinical history and current comorbidities-medications were collected. Each patient underwent a neurological examination to assess neurological involvement (Neuro-BD) and, if necessary, instrumental investigations. One hundred-fifty healthy subjects matched for age and gender were used as control group for comparison. Results: Of the 55 patients diagnosed as BD (ISG criteria) 41 patients adhered and were enrolled into the study. Headache occurred in 29 of BD patients (70,7%) and in 13 of Neuro-BD patients (92,8%). Migraine without aura did prove the most frequent type of headache in BD patients (with and without neurological involvement) and there were no differences in the frequency of the different pattern of headache between BD patients and controls. Conclusions: Headache is a frequent manifestation in BD and primary headache like migraine emerged as the most frequent type of headache. A careful search for headache should be included in the diagnostic work-up of BD since this manifestation may be related to the underlying disease

Autoinflammatory syndromes: inborn errors of natural immunity

The notion of autoinflammatory diseases delineates a heterogenous group of genetic pathologies characterized by spontaneous periodic systemic inflammation in the absence of infectious or autoimmune causes. The general hypothesis is that the innate immune response in these patients is wrongly tuned, being either too sensitive to minor stimuli or turned off too late. Clinical pictures of these disorders are characterized by high spiking fever associated with involvement of musculo-skeletal system, tegumentary apparatus and serosas. Although inflammatory syndromes are considered rare, they may represent a model in order to unravel some aspects of the innate immune system and of the inflammatory cascade

Headache in Behçet’s disease: case reports and literature review

Objective: To evaluate the prevalence of headache and its different patterns in patients with Behçet’s disease (BD) with and without neurological involvement and to investigate clinical correlations. Methods: Patients fulfilling the International Study Group criteria for Behçet disease (ISGc) were studied. Patients were invited to fill a “headache questionnaire”, which consisted of two sections: the first one included demographic and anamnestic data, family history for both headache and BD, disease duration and clinical manifestations of BD; the second section included items about headache, investigated accordingly to International Headache Society diagnostic criteria (IHS, 2004). Clinical history and current comorbidities-medications were collected. Each patient underwent a neurological examination to assess neurological involvement (Neuro-BD) and, if necessary, instrumental investigations. One hundred-fifty healthy subjects matched for age and gender were used as control group for comparison. Results: Of the 55 patients diagnosed as BD (ISG criteria) 41 patients adhered and were enrolled into the study. Headache occurred in 29 of BD patients (70,7%) and in 13 of Neuro-BD patients (92,8%). Migraine without aura did prove the most frequent type of headache in BD patients (with and without neurological involvement) and there were no differences in the frequency of the different pattern of headache between BD patients and controls. Conclusions: Headache is a frequent manifestation in BD and primary headache like migraine emerged as the most frequent type of headache. A careful search for headache should be included in the diagnostic work-up of BD since this manifestation may be related to the underlying disease

The diagnostic value of 99mTc-DTPA lung clearance and esophageal transit to evaluate the severity of complications in systemic sclerosis

Background: Lung and esophageal involvements are common in systemic sclerosis (SSc). Aim of this study is to assess if there is a correlation between the involvement of the respiratory and gastrointestinal systems in the same patients.Materials and Methods: We studied 280 patients effected by SSc (259 females and 14 males), with a mean age of 55 years (from 20 to 78 years old) and a mean lenght of disease of 106 months. All pts underwent 99mTc-DTPA clearance using a commercial available jet nebulizer (Venticis, CIS) aerosol inhaling 500 MBq of 99mTc-DTPA aerosol for 2 minutes at normal tiding breathing. Images were recorded dinamically with a dual head camera (Philips Vertex) and two ROIs were defined around lungs and the activity/time curves were generated. The best exponential fit of both curves in the first 15 minutes were calculated: the results were considered abnormal if half time was < 60 minutes. All patients underwent to esophageal transit scintigraphy with the assessment of esophageal transit time of liquid and solid swallows in upright and supine position (10 ml of water or fruit juice labelled with 40 MBq of 99mTc-colloid)

Colour Duplex Ultrasonography in the Diagnosis and Management of Giant Cell Arteritis

Though several studies have emphatized the diagnostic potentialities of color duplex sonography (CDS) in giant cell arteritis (GCA) bipsy remains the gold standard for the diagnosis. Recently it has been suggested that the dark hypoechogenic halo is strongly related to the acute phase of the disease so CDS of the temporal artery may be a useful tool in monitoring and assisting glucocorticoid tapering in patients with GCA. To date, acute phase reactants generally used for this purpose but they are not absolute indicators of disease activity. The aim of our study was to evaluate the role of color duplex ultrasonography in diagnosis and follow-up in patients with GC