Childhood onset of psoriatic onycho-pachydermo-periostitis (POPP).
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Adolescent↗
Biomedical subjects
P Manganelli
Publications and source records attributed to P Manganelli.
Contrast-enhanced power Doppler sonography of knee synovitis in rheumatoid arthritis: assessment of therapeutic response.
The aim of this study was to evaluate the ability of power Doppler sonography (PDS) with ultrasound contrast agent to assess the synovial perfusion changes induced by intra-articular steroid injection therapy in the knee joints of patients with rheumatoid arthritis (RA). Eighteen RA patients (16 women, 2 men) with a history and signs of active knee synovitis were studied. Tenderness was evaluated using Thompson's modified index of synovitis activity. All patients underwent joint aspiration followed by intra-articular injection of 40 mg of triamcinolone hexacetonide. Gray-scale ultrasonography and PDS with an intravenous ultrasound contrast agent (Levovist) examinations were carried out before and 3 weeks after the intra-articular steroid injection. The calculation of the time--intensity curves provided a quantitative estimation of the synovial perfusion. The median values of the index of synovitis activity decreased significantly from 7.0 (95% confidence interval (CI) 6.0-8.0) to 3.0 (95% CI 2.0-4.0) ( p<0.01) 3 weeks after the intra-articular steroid injection. All patients showed a reduction of PDS signal after intra-articular steroid therapy and the baseline and follow up median values of the area underlying time-intensity curves were 7.48 (95% CI 5.79-8.73) and 2.45 (95% CI 1.92-3.61), respectively. The comparison between baseline and follow-up median values of the area under the curves showed a statistically significant reduction of PDS findings ( p<0.01). At follow-up examinations the changes in the index score of the synovitis activity were significantly correlated to the changes in the values of the area underlying time-intensity curves ( r=0.785; p<0.01). A significant correlation was also observed between baseline values of the area underlying time-intensity curves and C-reactive protein (CRP) ( r=0.548; p=0.023). In conclusion, PDS with an intravenous ultrasound contrast agent has been shown to be able to detect changes in synovial perfusion after intra-articular steroid injection and may be an additional useful method in the evaluation of synovial inflammation and in the assessment of the therapeutic response.
Adult↗
Pachydermoperiostosis and psoriatic onychopathy: an unusual association.
A 33-year-old man, suffering from cutaneous psoriasis since the age of 16, in the last 6 years experienced slow and painless enlargement at his fingertips and later at his big toes, which resulted in digital clubbing. Since the age of 31, the patient also presented psoriatic nail changes involving all his fingernails, without joint pain or inflammation. The patient's family history was negative for psoriasis, however, his brother, a 29-year-old healthy man, also presented digital clubbing. The diagnosis of pachydermoperiostosis coexistent with ungual and cutaneous manifestations of psoriasis vulgaris was made. The differential diagnosis with psoriatic onycho-pachydermo-periostitis, as well as other clinical conditions that involve the distal interphalangeal joints is discussed.
Adult↗
[The hemophagocytic syndrome (macrophage activation syndrome)].
The hemophagocytic syndrome (HPS) is an uncommon, often misdiagnosed life-threatening disorder of immune regulation, characterized by a widespread proliferation and multisystemic infiltration of non-malignant histiocytes that undergo uncontrolled hemophagocytosis in bone marrow and/or reticulo-endothelial system. The HPS immune dysfunction consists in a low or absent cytotoxic T and natural killercell activity and in hyperactivation of T lymphocytes and macrophages, with consequent proinflammatory cytokine storm. Clinically, HPS is characterized by high fever, lymphadenopathies, hepato-splenomegaly, liver dysfunction, (pan)cytopenia, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, as well as coagulopathy and neurological manifestations in many cases. A hereditary/primary variant (familial hemophagocytic lymphohistiocytosis) and an acquired/secondary form (reactive HPS) are known. This latter may occur in several disorders, including infections, immunodeficiency states, malignancies, lymphoproliferative and autoimmune diseases. Without treatment, HPS fatally has an unfavourable prognosis. Recently, outcome improvements have been reported, due to better therapeutic strategies. The clinical and pathological features of this syndrome are reviewed.
Histiocytosis, Non-Langerhans-Cell↗
Power Doppler sonography in the assessment of synovial tissue of the knee joint in rheumatoid arthritis: a preliminary experience.
OBJECTIVE: To investigate the intra-articular vascularisation of the synovial pannus in the knee of patients with rheumatoid arthritis (RA) with power Doppler ultrasonography (PDS) and an echo contrast agent and correlate the area under the time-intensity curves with the clinical findings and laboratory measures of disease activity. METHOD: Forty two patients with RA (31 women, 11 men) with history and signs of knee arthritis, classified according to a modified index of synovitis activity (active, moderately active, and inactive), were studied. Clinical and functional assessment (number of swollen joints, intensity of pain, general health-visual analogue scale, disability index-Health Assessment Questionnaire, Ritchie articular index) and a laboratory evaluation were made on all patients. Disease activity was evaluated using the disease activity score (DAS) and the chronic arthritis systemic index (CASI) for each patient. All patients were examined with conventional ultrasonography and PDS before injection of intravenous ultrasound contrast agent (Levovist). The quantitative estimation of the vascularisation of the synovial membrane was performed with time-intensity curves and calculation of the area under the curves. RESULTS: The mean (SD) value of the area underlying time-intensity curves was 216.2 (33.4) in patients with active synovitis, 186.8 (25.8) in patients with moderately active synovitis, and 169.6 (20.6) in those with inactive synovitis. The mean value of the areas differed significantly between the patients with active and those with inactive synovitis (p<0.01). The mean value of the area under the curve of the entire group was weakly correlated with the number of swollen joints (p=0.038), but a strong correlation was found with composite indexes of disease activity such as the DAS (p=0.006) and CASI (p=0.01). No correlation was found with age, disease duration, and other laboratory and clinical variables. CONCLUSION: PDS may be a valuable tool to detect fractional vascular volume and to assist clinicians in distinguishing between inflammatory and non-inflammatory pannus. The transit of microbubbles of ultrasound contrast across a tissue can be used to estimate haemodynamic alterations and may have a role in assessing synovial activity and the therapeutic response to treatment of synovitis of the knee joint.
Adult↗
[Adult onset Still's disease].
Adult onset Still's disease (AOSD), the adult variant of the systemic form of the juvenile rheumatoid arthritis, is an uncommon disorder of unknown origin. Although the pathogenesis has not yet been clarified, an immunologically mediated inflammation occurs in active AOSD. High spiking fever, evanescent maculo-papular skin rash, arthralgias/arthritis, neutrophilic leukocytosis, negative rheumatoid factor and antinuclear antibodies, as well as a marked hyperferritinemia are the major features of AOSD. Sore throat, lymphadenopathies, hepato-splenomegaly, abdominal pain, polyserositis, respiratory distress syndrome, multiple organ dysfunction and disseminated intravascular coagulation may also occur. The clinical course of AOSD is extremely variable and unpredictable and can be divided into three main patterns: a self-limited or monocyclic pattern, a polycyclic or intermittent course, with one or more flares of the disease and complete remission among the episodes, and a chronic course, characterized by persistently active disease, usually due to a chronic, destructive arthritis. Since there are not pathognomonic laboratory parameters or histological findings, the diagnosis of AOSD requires the exclusion of infectious, malignant and autoimmune disorders. Some sets of criteria for classification have been proposed, but so far not validated. The prognosis of AOSD is usually considered relatively benign, although a destructive arthritis may cause severe disability and the multisystemic life-threatening complications of the disease may determine a fatal outcome. Treatment usually consists in nonsteroidal anti-inflammatory drugs and corticosteroids, but a more aggressive approach with disease modifying antirheumatic or immunosuppressive drugs may be required.
Adult↗
Decreased DHEAS secretion in patients with chronic inflammatory diseases treated with glucocorticoids.
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Adult↗
[Mondor's diseases. Spectrum of the clinical and pathological features].
Mondor's disease is an uncommon disorders, occurring mostly in middle-aged women, and characterized by superficial thrombophlebitis classically involving the thoraco-epigastric veins and/or their confluents. Rare cases have been reported in atypical sites (upper arms, abdomen, groin and penis). The most common clinical features include lateral chest wall tension and pain, as well as the presence of a tender subcutaneous linear or winding cord-like structure, corresponding to the affected vessel, often with skin redness, edema or retraction. The etiology is unknown. Mondor's disease may be primary or secondary to local trauma, surgical procedures, bandaging, tight clothes and infections; it may also be associated with breast cancer. This condition is usually a benign and self-limited process, requiring only symptomatic treatment. Surgery is indicated when it is associated with malignancies or severe local pain and retraction. The clinical and pathological spectrum of this disease are reviewed.
Humans↗
Remitting seronegative symmetrical synovitis with pitting oedema in a patient with myelodysplastic syndrome and relapsing polychondritis.
Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) may be the inaugural manifestation of different rheumatic diseases of the elderly, malignancies and myelodysplastic syndromes (MDS). Relapsing polychondritis (RP) is a rare systemic disorder characterised by an inflammatory process involving predominantly cartilaginous structures, the cardiovascular system and organs of special sense. We report on a 72-year-old man with RS3PE and MDS, refractory anaemia subtype, diagnosed at the same time as RS3PE. Several months later the patient presented a clinical and pathological picture compatible with RP. Although the association between RP and MDS is well known, no previous cases of RS3PE preceding RP have been reported. This case confirms that RS3PE may herald many diseases, among others autoimmune disorders such as RP.
Aged↗
Ultrasonography and colour doppler sonography of salivary glands in primary Sjögren's syndrome.
To examine either the ultrasonographic (US) features of the parotids and submandibular glands or the blood flow alterations that may occur in the salivary glands of patients with primary Sjögren's syndrome (pSS) we studied 30 female patients with pSS and 30 controls suffering from dry mouth not due to pSS. All measurements were taken by the same examiner, who used the same equipment to avoid interobserver variability. The US parameters recorded (parenchymal homogeneity, echogenicity, size of the glands and posterior glandular border) were scored according to a previously described scoring system. For each waveform, peak systolic velocity (PSV) and resistive index (RI) were measured at the external carotid artery in the examination of the parotids and at the facial artery within the submandibular glands before and during lemon juice stimulation. On the basis of the degree of chronic inflammatory changes at minor salivary gland (MSG) biopsy, chronic sialadenitis (CS) was defined as mild in 10 and severe in 20 pSS patients. Abnormal US scores were obtained in 26/30 (86.6%) pSS patients and in 9/30 (30%) controls. Moreover, in pSS patients the US scores were sigificantly higher than in the control group (p=-0.0001). The mean (+/- SD) difference between the PSV values taken from parotids and submandibular glands before and during lemon juice stimulation was statistically significant (p=0.003 and p=0.01, respectively) in the controls. On the other hand, no significant changes in the PSV values were found in the whole group of pSS patients. However, the changes in PSV values before and during lemon juice stimulation were statistically significant in both parotids (p=0.019) and submandibular glands (p=0.012), and not significantly different from those in the controls in pSS patients with mild CS. The variability of RI taken from the salivary glands before and during lemon juice stimulation was not statistically significant in either pSS patients or controls. US abnormalities were detected in the majority of pSS patients and their severity was significantly greater than those recorded in the controls. Of the colour Doppler sonographic (CDS) parameters only PSV was influenced by the degree of chronic inflammation, as shown at the MSG biopsy, suggesting that PSV may reflect the vascular changes occurring in the salivary glands during the course of an autoimmune disease such as pSS.
Adult↗
Coexistent Marfan's syndrome and ankylosing spondylitis: a case report.
We report on a 46-year-old man with a 4-year history of predominantly nocturnal pain at the thoracic and lumbar spine as well as accompanying morning stiffness and episodes of alternating buttock pain. At physical examination the patient presented with the typical traits for Marfan's syndrome (MFS), along with limitation of both chest expansion and movement in all planes of the lumbar spine. Pelvic and lumbar spine radiographs showed findings consistent with ankylosing spondylitis (AS). Laboratory tests were consistent with an inflammatory state and HLA typing was positive for the B27 antigen. Transthoracic echocardiography showed prolapse of the posterior mitral leaflet and mild aortic insufficiency. We diagnosed co-existent MFS and AS. The association of these two pathologies is particularly interesting, owing to the co-existence of hypermobility of peripheral joints due to MFS ligamentous hyperlaxity, and the reduction of both axial skeleton motility and chest expansion related to AS. As both of these diseases may damage the cardiovascular system over time, follow-up with echocardiography monitoring is indispensable.
Echocardiography↗
Interferon-alpha in combination with ribavirin as initial treatment for hepatitis C virus-associated cryoglobulinemic membranoproliferative glomerulonephritis.
Mixed cryoglobulinemia (MC) and glomerulonephritis are the most important extrahepatic manifestations of chronic hepatitis C virus (HCV) infection. In HCV-infected patients with MC, renal involvement worsens the overall prognosis because of a high incidence of infection or cardiovascular disease. The relationship between MC and HCV infection has prompted the use of antiviral therapy. Two patients with chronic HCV infection, type-II MC and membranoproliferative glomerulonephritis (MPGN), presenting as nephrotic syndrome were treated with interferon (IFN)-alpha (3 MU 3 times per week) and ribavirin (15 mg/kg daily) for 6 months. Laboratory tests included measurement of anti-HCV antibodies, HCV RNA, and HCV genotyping, and characterization of circulating cryoglobulins. A pretreatment renal biopsy was performed, and the histopathologic lesions were scored according to the index of disease activity. Viremia and cryoglobulinemia were suppressed in both patients. However, a complete remission of proteinuria was observed in 1 patient only. The evaluation of the renal biopsy specimens revealed a mild MPGN (activity score: 5/24) in the patient with remission of proteinuria and a severe MPGN (activity score: 15/24) in the patient who maintained a nephrotic-range proteinuria. Although a fully satisfactory treatment is not yet available, we feel that a reasonable therapeutic strategy for HCV-infected patients with MC nephritis could be as follows: (1) antiviral treatment alone for patients with a low-grade kidney involvement, and (2) a short-term course of steroids and cytotoxic drugs followed by antiviral therapy for acute exacerbations and/or rapidly progressive GN.
Adult↗
Mondor's disease and polymyalgia rheumatica: a case report.
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Letter↗
[Cryoglobulinemia and hepatitis C virus infection].
The demonstration of the high prevalence of HCV infection (HCV) in patients with MC has changed the clinico-biologic scenario of MC, supporting its subdivision into two groups: MC HCV- and MC HCV+. The former, which is predominantly a polyclonal cryoglobulinemia, should be regarded as an epiphenomenon of the immune system activation in the course of a variety of chronic infections or autoimmune disorders; the latter, which is a oligo- or monoclonal cryoglobulinemia, referred in the past as "essential mixed cryoglobulinemia", might be expression of an indolent B cell proliferation stimulated by HCV in an antigen-driven mechanism. The association of HCV infection with MC may have a pathogenetic an therapeutic significance. There are a number of reports demonstrating the beneficial effects of alpha-interferon (alpha-IFN) in about a half of patients with chronic HCV and MC. However, after the end of alpha-IFN therapy a recurrence of viremia and cryoglobulinemia is frequently observed and less than 25% of treated patients achieve long term remissions. To improve the sustained response rate, prolonged courses of alpha-IFN monotherapy or a combination of alpha-IFN and ribavirin should be considered. New agents with specific antiviral activity against HCV will probably further improve therapeutic options.
Cryoglobulinemia↗
Severe glomerulonephritis and isolated ileitis in adult Henoch-Schoenlein purpura.
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Anti-Infective Agents↗
The differing patterns of subclinical pulmonary involvement in connective tissue diseases as shown by application of factor analysis.
To explore common patterns of interstitial lung disease (ILD) in symptomless patients with connective tissue disease (CTD), we applied factorial analysis to determine the relationship among the factors. A selected cohort of 71 non-smoking patients with a confirmed diagnosis of CTD [24 with primary Sjögren's syndrome (pSS), 21 with systemic sclerosis (SS), 20 with rheumatoid arthritis (RA) and six with polymyositis/ dermatomyositis (PM/DM)] were identified. The diagnostic techniques included pulmonary function tests, bronchoalveolar lavage (BAL), chest radiographs and high-resolution computed tomography (HRCT). Disease extent and severity were assessed by a radiological and HRCT grading system. Three factors, accounting for 67% of the total variance, were extracted. The first factor (disease duration, diffusing lung capacity, neutrophils and CD8+ T cells on BAL, radiographic score and HRCT reticular score), with the highest percentage of variance (36.5%), defines a fibrotic lung pattern. The second factor (17.9% of variance) identifies an inflammatory lung pattern (macrophages, lymphocytes and eosinophils on BAL and HRCT ground-glass score). The third factor (12.6% of variance) represents a ventilatory function pattern (forced vital capacity, total lung capacity and forced respiratory volume in 1 s). The negative correlation between the fibrotic lung pattern and ventilatory function pattern, but not with the inflammatory lung pattern, suggests the presence of a significant derangement of the alveolar structures. In conclusion, application of factor analysis reveals various lung disease patterns in patients with CTD that might have different prognostic implications.
Adult↗
[Pulmonary hypertension in rheumatic diseases].
In rheumatic diseases (RD) pulmonary hypertension (PH) may result by either direct damage of the pulmonary arteries (isolated PH) or pulmonary interstitial fibrosis and other causes. PH is an important cause of morbidity and mortality in systemic sclerosis in which it is more frequently isolated in the limited cutaneous variant and secondary to interstitial fibrosis in the diffuse type. In isolated PH the main histopathological finding is an occlusive arteriopathy. The role of recurrent vasospasm ("lung Raynaud's phenomenon") is still being debated. In systemic lupus erythematosus, although uncommon, PH is being increasingly reported and may recognize multiple etiological factors including vasoconstriction, vasculitis, in-situ pulmonary thrombosis or chronic recurrent thromboembolism. PH may be a severe and often fatal complication of mixed connective tissue disease and dermato/polymyositis. PH may also be diagnosed in patients with rheumatoid arthritis, primary Sjögren's syndrome and primary antiphospholipid syndrome. Doppler echocardiography is the technique of choice for the evaluation of PH because it is nonivasive and allows serial determinations of the arterial pulmonary pressure. The therapy of PH associated with RD includes corticosteroids, immunosuppressive drugs, calcium-antagonists, ACE-inhibitors, anticoagulants, O2, prostacyclin or its stable analogue, iloprost. Carefully selected patients may benefit from single lung or heart-lung transplantation.
Algorithms↗