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1.
Eur Ann Allergy Clin Immunol ; 49(4): 154-160, 2017 Jul.
Article En | MEDLINE | ID: mdl-28752718

SUMMARY: Background. Histamine release (HR) test has previously been shown to predict the presence of endogenous histamine-releasing factors in chronic spontaneous urticaria (CSU). Objectives and methods. Twenty CSU patients unresponsive to antihistamine treatment were enrolled in order to evaluate the correlations between HR test results and demographic features, quality of life, disease activity, clinical course, and autologous serum and plasma skin tests (ASST and APST). Results. All patients with positive HR test (9/9, 100%) had a more severe disease activity at onset (urticaria activity score, UAS > 2) when compared to negative HR test patients (5/11; p = 0.04). Quality of life questionnaire's results were not substantially different between HR positive and negative subgroups at baseline (p > 0.05), and results of HR test and ASST/APST did not co-segregate (p > 0.05). After 12 months, patients with a positive HR test had a significant reduction of disease activity (p = 0.003) whereas patients with a negative HR test did not (p > 0.05), leading to disease remission and antihistamine treatment withdrawal in 67% (6/9) of positive HR test patients versus 18% (2/11) of negative HR test patients (p = 0.027). Conclusions. Positive HR test may predict spontaneous CSU remission at 12 months.


Histamine Antagonists/therapeutic use , Histamine Release/drug effects , Immunologic Tests , Mast Cells/drug effects , Urticaria/diagnosis , Urticaria/drug therapy , Adolescent , Adult , Aged , Chronic Disease , Female , Humans , Male , Mast Cells/immunology , Mast Cells/metabolism , Middle Aged , Phenotype , Predictive Value of Tests , Proof of Concept Study , Prospective Studies , Quality of Life , Remission Induction , Severity of Illness Index , Surveys and Questionnaires , Time Factors , Treatment Outcome , Urticaria/immunology , Urticaria/metabolism , Young Adult
2.
Eur Ann Allergy Clin Immunol ; 48(4): 137-44, 2016 Jul.
Article En | MEDLINE | ID: mdl-27425169

BACKGROUND: The term "breakthrough reactions" designates repeated hypersensitivity reactions to iodinated contrast media (ICM) despite premedication with glucocorticoids and antihistamines. We aimed to retrospectively evaluate the rate of positive skin test (STs) in our cohort of patients with previous breakthrough reactions to different ICMs. METHODS: A series of 35 patients, who experienced at least one breakthrough reaction to ICM and who underwent STs within 6 months from the reaction were studied, and results were compared to a control group of patients with a first hypersensitivity reaction occurred without premedication. Skin prick tests (SPT), intradermal tests (IDT) and patch tests (PT) at different dilutions, with a set of three to four ICM were performed. RESULTS: Of the 35 patients with prior breakthrough reactions, 57% had an immediate reaction (IR) and 43% had a non-immediate reaction (NIR). Patients who experienced the first hypersensitivity IR or NIR, later had one or more breakthrough IR or NIR, respectively. Overall, 29% (10/35) of patients with prior breakthrough reactions resulted positive to STs compared to 57% (16/28) of the control group (p < 0.05). No significant difference in allergy history, age, sex, other clinical / demographic features nor chronic use of ACE-inhibitor, beta-blockers or NSAIDs was observed. CONCLUSION: This preliminary finding suggests that patients with prior breakthrough reactions have significantly lower immunologically proven ICM reactions (positive STs) if compared to non-breakthrough patients. According to that, a considerable number of breakthrough reactions seems to be non-allergic hypersensitivity reactions or reactions which could be mostly prevented by a proper, well-timed skin testing. Larger prospective studies are needed to confirm these results, with a more careful analysis of patients' risk factors, a laboratory assessment that includes an in vitro allergy diagnostics, and hopefully a drug provocation test for selected cases.


Contrast Media/administration & dosage , Drug Hypersensitivity/diagnosis , Hypersensitivity, Delayed/diagnosis , Hypersensitivity, Immediate/diagnosis , Intradermal Tests , Patch Tests , Adult , Aged , Drug Hypersensitivity/immunology , Drug Hypersensitivity/prevention & control , Female , Glucocorticoids/therapeutic use , Histamine Antagonists/therapeutic use , Humans , Hypersensitivity, Delayed/chemically induced , Hypersensitivity, Delayed/immunology , Hypersensitivity, Delayed/prevention & control , Hypersensitivity, Immediate/chemically induced , Hypersensitivity, Immediate/immunology , Hypersensitivity, Immediate/prevention & control , Male , Middle Aged , Predictive Value of Tests , Recurrence , Reproducibility of Results , Retrospective Studies , Risk Factors
3.
Scand J Rheumatol ; 45(2): 135-45, 2016.
Article En | MEDLINE | ID: mdl-26398142

OBJECTIVES: To describe the clinical features, treatment response, and follow-up of a large cohort of Italian patients with immunoglobulin (Ig)G4-related disease (IgG4-RD) referred to a single tertiary care centre. METHOD: Clinical, laboratory, histological, and imaging features were retrospectively reviewed. IgG4-RD was classified as 'definite' or 'possible' according to international consensus guidelines and comprehensive diagnostic criteria for IgG4-RD. Disease activity was assessed by means of the IgG4-RD Responder Index (IgG4-RD RI). RESULTS: Forty-one patients (15 females, 26 males) were included in this study: 26 with 'definite' IgG4-RD and 15 with 'possible' IgG4-RD. The median age at diagnosis was 62 years. The median follow-up was 36 months (IQR 24-51). A history of atopy was present in 30% of patients. The pancreas, retroperitoneum, and major salivary glands were the most frequently involved organs. Serum IgG4 levels were elevated in 68% of cases. Thirty-six patients were initially treated with glucocorticoids (GCs) to induce remission. IgG4-RD RI decreased from a median of 7.8 at baseline to 2.9 after 1 month of therapy. Relapse occurred in 19/41 patients (46%) and required additional immunosuppressive drugs to maintain long-term remission. Multiple flares occurred in a minority of patients. A single case of orbital pseudotumour did not respond to medical therapy and underwent surgical debulking. CONCLUSIONS: IgG4-RD is an elusive inflammatory disease to be considered in the differential diagnosis of isolated or multiple tumefactive lesions. Long-term disease control can be achieved with corticosteroids and immunosuppressive drugs in the majority of cases.


Autoimmune Diseases/immunology , Glucocorticoids/therapeutic use , Immunoglobulin G/immunology , Immunosuppressive Agents/therapeutic use , Pancreatitis/immunology , Sialadenitis/immunology , Aged , Autoimmune Diseases/complications , Autoimmune Diseases/drug therapy , Cohort Studies , Cytoreduction Surgical Procedures , Female , Humans , Italy , Male , Middle Aged , Orbital Pseudotumor/etiology , Orbital Pseudotumor/immunology , Orbital Pseudotumor/surgery , Pancreatitis/drug therapy , Pancreatitis/etiology , Recurrence , Remission Induction , Retroperitoneal Space , Retrospective Studies , Sialadenitis/drug therapy , Sialadenitis/etiology
4.
Eur Ann Allergy Clin Immunol ; 47(3): 77-85, 2015 May.
Article En | MEDLINE | ID: mdl-25951145

The purpose of the present work is to evaluate the efficacy of an approach that combines clinical history, skin tests results, and premedication, in preventing recurrent hypersensitivity reactions to iodinated contrast media (ICM). Skin Prick tests, Intradermal tests, and Patch tests were performed in 36 patients with a previous reaction to ICM. All patients underwent a second contrast enhanced radiological procedure with an alternative ICM selected on the basis of the proposed approach. After alternative ICM re-injection, only one patient presented a mild NIR. The proposed algorithm, validated in clinical settings where repeated radiological exams are needed, offers a safe and practical approach for protecting patients from recurrent hypersensitivity reactions to ICM.


Contrast Media/adverse effects , Drug Hypersensitivity/prevention & control , Iodine Compounds/adverse effects , Skin Tests , Adult , Aged , Algorithms , Critical Pathways , Drug Hypersensitivity/diagnosis , Drug Hypersensitivity/etiology , Female , Humans , Intradermal Tests , Male , Middle Aged , Predictive Value of Tests , Recurrence , Reproducibility of Results , Risk Factors , Young Adult
5.
Scand J Rheumatol ; 44(4): 309-14, 2015.
Article En | MEDLINE | ID: mdl-25656459

OBJECTIVES: To describe the efficacy and safety of different biological agents in a large cohort of 20 patients with adult-onset Still's disease (AOSD). METHOD: We retrospectively evaluated 20 patients with severe or refractory AOSD treated with at least one biological agent (anakinra, etanercept, tocilizumab, and adalimumab), followed up for at least 12 months at our Institution. We collected and analysed data on the disease course, treatment outcome, and adverse effects, and compared our data with other published series. RESULTS: The median duration of follow-up was 5 years. In 12 patients a single biological drug induced a clinical response. In eight patients the biological agent that was first administered proved ineffective, and a switch to a different biologic was necessary. In three patients a third biologic was necessary to achieve disease control. The biologics eventually determined a clinical response in all patients. Patients with systemic disease showed better responses than patients with chronic articular disease (p < 0.05). Biological agents allowed either the withdrawal or the tapering of corticosteroid therapy (p < 0.0001) and of disease-modifying anti-rheumatic agents (DMARDs; p < 0.05). Three patients experienced herpes zoster reactivation. CONCLUSIONS: This is the longest follow-up of a cohort of AOSD patients treated with biological agents. Our data show that biologics are safe and generally effective in the long-term management of AOSD, particularly in cases with systemic disease, and suggest that a clinical response can be obtained in almost all AOSD patients, although a switch to drugs with a different mechanism of action may be necessary.


Biological Factors/adverse effects , Biological Factors/therapeutic use , Still's Disease, Adult-Onset/drug therapy , Tumor Necrosis Factor-alpha/antagonists & inhibitors , Adalimumab , Adolescent , Adult , Aged , Antibodies, Monoclonal, Humanized/adverse effects , Antibodies, Monoclonal, Humanized/therapeutic use , Cohort Studies , Etanercept , Female , Follow-Up Studies , Humans , Immunoglobulin G/adverse effects , Immunoglobulin G/therapeutic use , Interleukin 1 Receptor Antagonist Protein/adverse effects , Interleukin 1 Receptor Antagonist Protein/therapeutic use , Longitudinal Studies , Male , Middle Aged , Receptors, Tumor Necrosis Factor/therapeutic use , Retrospective Studies , Treatment Outcome , Young Adult
6.
Infection ; 43(3): 367-70, 2015 Jun.
Article En | MEDLINE | ID: mdl-25575464

Legionella-associated pancreatitis has been rarely reported. Since this condition is very rarely suspected and investigated in patients with Legionella pneumonia, its incidence is probably underestimated. Here we report a case of Legionella pneumonia-associated pancreatitis and review the relevant related literature.


Legionnaires' Disease/complications , Pancreatitis/diagnosis , Pancreatitis/pathology , Aged, 80 and over , Female , Humans , Legionella pneumophila/isolation & purification , Radiography, Thoracic
7.
Nutr Metab Cardiovasc Dis ; 24(7): 751-9, 2014 Jul.
Article En | MEDLINE | ID: mdl-24787906

BACKGROUND AND AIM: Patients with systemic lupus erythematosus (SLE) have a higher prevalence of subclinical atherosclerosis and higher risk of cardiovascular (CV) events compared to the general population. The relative contribution of CV-, immune- and disease-related risk factors to accelerated atherogenesis in SLE is unclear. METHODS AND RESULTS: Fifty SLE patients with long-lasting disease (mean age 44 ± 10 years, 86% female) and 50 sex- and age-matched control subjects were studied. Common carotid artery intima-media thickness (CCA-IMT) was used as a surrogate marker of atherosclerosis. We evaluated traditional and immune- and disease-related factors, assessed multiple T-cell subsets by 10-parameter-eight-colour polychromatic flow cytometry and addressed the effect of pharmacological therapies on CCA-IMT. In SLE patients, among several cardiometabolic risk factors, only high-density lipoprotein levels (HDL) and their adenosine triphosphate-binding cassette transporter 1 (ABCA-1)-dependent cholesterol efflux capacity were markedly reduced (p < 0.01), whereas the CCA-IMT was significantly increased (p = 0.03) compared to controls. CCA-IMT correlated with systolic blood pressure, low-density lipoprotein (LDL) cholesterol and body mass index (BMI), but not with disease activity and duration. The activated CD4(+)HLA-DR(+) and CCR5(+) T-cell subsets were expanded in SLE patients. Patients under hydroxychloroquine (HCQ) therapy showed lower CCA-IMT (0.62 ± 0.08 vs. 0.68 ± 0.10 mm; p = 0.03) and better risk-factor profile and presented reduced circulating pro-atherogenic effector memory T-cell subsets and a parallel increased percentage of naïve T-cell subsets. CONCLUSION: HDL represents the main metabolic parameter altered in SLE patients. The increased CCA-IMT in SLE patients may represent the net result of a process in which 'classic' CV risk factors give a continuous contribution, together with immunological factors (CD4(+)HLA-DR(+) T cells) which, on the contrary, could contribute through flares of activity of various degrees over time. Patients under HCQ therapy present a modified metabolic profile, a reduced T-cell activation associated with decreased subclinical atherosclerosis.


Cardiovascular Diseases/blood , Carotid Artery, Common/physiopathology , Carotid Intima-Media Thickness , Immunologic Factors/metabolism , Lupus Erythematosus, Systemic/blood , ATP Binding Cassette Transporter 1/blood , Adult , Biomarkers/blood , Blood Pressure/drug effects , Body Mass Index , CD4-Positive T-Lymphocytes/metabolism , Cardiovascular Diseases/drug therapy , Carotid Artery, Common/drug effects , Case-Control Studies , Cholesterol, HDL/blood , Cholesterol, LDL/blood , Female , Humans , Hydroxychloroquine/therapeutic use , Logistic Models , Lupus Erythematosus, Systemic/drug therapy , Male , Middle Aged , Multivariate Analysis , Risk Factors
8.
Clin Exp Immunol ; 175(2): 150-66, 2014 Feb.
Article En | MEDLINE | ID: mdl-24128276

Vascular inflammation contributes to the defence against invading microbes and to the repair of injured tissues. In most cases it resolves before becoming apparent. Vasculitis comprises heterogeneous clinical entities that are characterized by the persistence of vascular inflammation after it has served its homeostatic function. Most underlying mechanisms have so far remained elusive. Intravascular immunity refers to the surveillance of the vasculature by leucocytes that sense microbial or sterile threats to vessel integrity and initiate protective responses that entail most events that determine the clinical manifestations of vasculitis, such as end-organ ischaemia, neutrophil extracellular traps generation and thrombosis, leucocyte extravasation and degranulation. Understanding how the resolution of vascular inflammation goes awry in patients with systemic vasculitis will facilitate the identification of novel pharmacological targets and bring us a step closer in each patient to the selection of more effective and less toxic treatments.


Blood Vessels/immunology , Systemic Vasculitis/immunology , Systemic Vasculitis/microbiology , B-Lymphocytes/immunology , Bacterial Infections/immunology , Blood Vessels/pathology , C-Reactive Protein/immunology , Humans , Inflammasomes/immunology , Serum Amyloid P-Component/immunology , T-Lymphocytes/immunology , Thrombosis/immunology , Thrombosis/pathology
9.
Lupus ; 21(7): 810-2, 2012 Jun.
Article En | MEDLINE | ID: mdl-22635241

The impact of hypertension in the pregnancies from autoimmune patients is not unequivocally defined. We have prospectively followed 168 pregnancies from 135 patients from four Italian centres to verify the potential impact of hypertension in the antiphospholipid syndrome (APS). The rate of preeclampsia, mean neonatal weight and gestational age at delivery were significantly lower in patients with both APS and hypertension than in patients with hypertension or APS alone. This information may be relevant for counselling and care of these patients.


Antiphospholipid Syndrome/complications , Hypertension, Pregnancy-Induced/epidemiology , Adult , Antiphospholipid Syndrome/epidemiology , Birth Weight , Female , Gestational Age , Humans , Infant, Newborn , Italy/epidemiology , Pre-Eclampsia/epidemiology , Pregnancy , Pregnancy Outcome , Prospective Studies
10.
Clin Exp Rheumatol ; 28(1): 41-8, 2010.
Article En | MEDLINE | ID: mdl-20346237

OBJECTIVES: To characterise the clinical phenotype of Italian patients with adult-onset Still's disease (AOSD). METHODS: Sixty-six subjects who received a definite diagnosis of AOSD were seen and followed-up at our institution from 1991 to 2009. The diagnosis was made by a senior rheumatologist and confirmed by Yamaguchi's criteria for AOSD. Data regarding clinical manifestations, laboratory and radiographic features, and disease course were collected and compared with those reported in other published series of different ethnicity. RESULTS: The most frequent features were: articular pain (100%), acute phase reactants elevation (100%), elevated serum ferritin (97%), high fever (95%), negative RF and ANA (92%), neutrophilia (82%), skin rash (79%), and overt arthritis (79%). Forty-percent of patients showed a chronic articular disease. Five subjects (8%) experienced severe, life-threatening complications, and 1 patient died. As compared to other North American, North European, Middle Eastern, and Far Eastern cohorts, Italian patients showed significant differences in several epidemiologic, clinical and laboratory features. CONCLUSIONS: Our data show that AOSD is rare in the Italian population, and that its clinical presentation appears to be significantly influenced by the ethnicity of the affected patients. Given its broad differential diagnosis, early recognition of this condition is challenging, but it could become crucial in the setting of severe complications. Beyond the protean manifestations of this disease, a clinical picture of seronegative febrile arthritis and skin rash, concurrent with a marked elevation in serum ferritin should always be mindful of AOSD.


Asian People/statistics & numerical data , Still's Disease, Adult-Onset/complications , Still's Disease, Adult-Onset/ethnology , White People/statistics & numerical data , Acute-Phase Proteins/metabolism , Adolescent , Adult , Aged , Antibodies, Antinuclear/blood , Female , Ferritins/blood , Fever/ethnology , Fever/physiopathology , Humans , Italy/epidemiology , Joints/physiopathology , Male , Middle Aged , Pain/ethnology , Pain/etiology , Pain/physiopathology , Rheumatoid Factor/blood , Still's Disease, Adult-Onset/physiopathology , Young Adult
11.
Eur Ann Allergy Clin Immunol ; 41(2): 50-5, 2009 Apr.
Article En | MEDLINE | ID: mdl-19585860

BACKGROUND: Subjects with drug hypersensitivity are sometimes simultaneously reactive to several drugs. This nosological entity is defined as multiple drug hypersensivity (MDH). Urticaria and angioedema are the commonest clinical manifestations of hypersensitivity drug reactions (HDR). These clinical signs are also pathognomonic of chronic idiopathic urticaria (CIU), whose pathogenetic mechanisms are still largely unknown. The diagnostic algorithm of CIU includes autologous serum skin test (ASST) and autologous plasma skin test (APST), which demonstrated a high positive and negative predictive value, in multiple nonsteroidal anti-inflammatory drugs (NSAIDs) intolerance. OBJECTIVE: to explore the underlying mechanism of MDH and to assess the correlation between such tests and autoimmune diseases (AD). METHODS: Twenty eight subjects with MDH referred to our Allergy/Immunology Unit were enrolled from May 2006 to May 2007. Eight healthy subjects served as controls. In addition to common diagnostic tools used in the diagnostic algorithm of MDH, enrolled subjects also underwent ASST and APST. RESULTS: Patients were predominantly female (23 female vs. 5 male; mean age 52.2 years). In 61% of cases MDH was associated with either CIU or AD. NSAIDs and antibiotics were the major causes of HIDR, both implied in 54% of subjects. The proportions of MDH-subjects with positive ASST and APST were 46.4% and 28.6%, respectively. All patients with MDH+AD+CIU (4/4) presented apositive ASST. CONCLUSIONS: In patients with MDH, ASST proved to be frequently positive, as previously described for multiple NSAIDs intolerance. In ASST-positive subjects, the activity of several drugs appears to add up FceRI-specific autoantibodies in the induction of the release of allergic mediators.


Autoimmune Diseases/complications , Drug Hypersensitivity/complications , Drug Hypersensitivity/etiology , Adolescent , Adult , Aged , Aged, 80 and over , Anti-Bacterial Agents/adverse effects , Anti-Bacterial Agents/immunology , Anti-Inflammatory Agents, Non-Steroidal/adverse effects , Anti-Inflammatory Agents, Non-Steroidal/immunology , Autoantibodies/immunology , Autoimmune Diseases/epidemiology , Autoimmune Diseases/immunology , Chronic Disease/epidemiology , Drug Hypersensitivity/epidemiology , Drug Hypersensitivity/immunology , Female , Hashimoto Disease/complications , Hashimoto Disease/epidemiology , Humans , Italy , Male , Middle Aged , Receptors, IgE/immunology , Serum/immunology , Skin Tests , Urticaria/complications , Urticaria/epidemiology , Young Adult
12.
Clin Exp Immunol ; 156(3): 395-404, 2009 Jun.
Article En | MEDLINE | ID: mdl-19309348

Vessel walls are the primary inflammatory sites in systemic vasculitides. In most cases the initiating event is unknown, and a self-sustaining circuit attracts and activates inflammatory leucocytes in the wall of vessels of various size and anatomical characteristics. Recent studies have revealed homeostatic roles of vascular inflammation and have identified the action of humoral innate immunity, in particular injury-associated signals and acute phase proteins, on the activation of circulating leucocytes, platelets and endothelial cells. These advances have provided clues to the molecular mechanisms underlying the vicious circle that maintains and amplifies vessel and tissue injury.


Vasculitis/immunology , Blood Platelets/physiology , C-Reactive Protein/immunology , Endothelium, Vascular/immunology , Humans , Platelet Activation/immunology , Serum Amyloid P-Component/immunology , Vasculitis, Leukocytoclastic, Cutaneous/immunology
14.
Clin Exp Rheumatol ; 25(3): 378-84, 2007.
Article En | MEDLINE | ID: mdl-17631733

OBJECTIVE: The aim of this study was to investigate potential risk factors for Sjögren's syndrome (SS) by means of a multi-centre case-control study, focusing in particular on familial and environmental risk factors. 140 female SS patients and 109 female controls with orthopaedic problems were consecutively enrolled in seven university hospitals in Italy. METHODS: Information regarding the patient's lifestyle, her medical, menstrual and pregnancy history, and any family history of autoimmune diseases (AD) was obtained through a detailed structured questionnaire. The odds ratio (OR) and 95% confidence interval (95%CI) were calculated using unconditional logistic regression, adjusting for age and family size. The probability of first-degree relatives developing an autoimmune disease was also investigated. RESULTS: A positive family history of AD was significantly associated with SS. Subjects with a first-degree relative (FDR) with AD showed a seven-fold increase in the risk for SS compared to controls (OR=7.4, 95%CI 2.8-20.1); the strength of this association increased with the number of relatives affected. Similarly, the FDR of SS patients had a higher risk of AD in comparison to subjects without FDR affected by SS. Women with one or more pregnancies had an increased risk of SS (OR=2.1, 95%CI 1.0-4.3). CONCLUSION: This study suggests that a family history of AD is associated with SS.


Reproductive History , Sjogren's Syndrome/genetics , Adult , Aged , Autoimmune Diseases/etiology , Case-Control Studies , Female , Health Surveys , Humans , Life Style , Logistic Models , Male , Middle Aged , Odds Ratio , Pedigree , Risk Factors , Sjogren's Syndrome/epidemiology , Smoking
15.
Clin Exp Rheumatol ; 24(2 Suppl 41): S60-4, 2006.
Article En | MEDLINE | ID: mdl-16859598

Meningeal involvement is a rare occurrence in Wegener's Granulomatosis (WG). A Medline search uncovered only 48 previously reported cases. Here we describe the clinical features of meningeal involvement in WG and to evaluate the association with systemic disease extension. Through a systematic literature review of papers concerning meningeal involvement in WG, we collected and analysed data about sex, age, disease extension, symptoms, cerebrospinal fluid examination, imaging, ANCA and histology about previously reported patients. Headache is almost always the first symptom of meningeal involvement in WG. Later in the course of the disease other abnormalities may develop. Among them cranial nerve palsy, seizures and encephalopathy are the most frequent. Diagnosis is obtained by neuroimaging, which may disclose two distinct patterns of meningeal thickening: diffuse or focal. 62.9% of patients tests positive for ANCA. Histology typically shows necrotizing granulomatosis. Meningeal involvement is by far more frequent in the setting of localized WG. Meningitis is a rare complication of WG. It usually develops in patients with localized disease who are more likely to have destructive lesions of the upper airways. It may be recognized by a constellation of clinical and radiological findings and by histological signs of necrotizing granulomatosis, with little or no vasculitis.


Granulomatosis with Polyangiitis/complications , Meninges/pathology , Meningitis/etiology , Adult , Antibodies, Antineutrophil Cytoplasmic/analysis , Cranial Nerve Diseases/etiology , Cranial Nerve Diseases/pathology , Female , Headache/etiology , Headache/pathology , Humans , Male , Meninges/chemistry , Meninges/diagnostic imaging , Meningitis/diagnosis , Meningitis/pathology , Middle Aged , Radiography , Seizures/etiology , Seizures/pathology
16.
Reumatismo ; 57(3): 187-92, 2005.
Article It | MEDLINE | ID: mdl-16258603

OBJECTIVES: To evaluate the rate of different organs involvement in 50 patients with Wegener's Granulomatosis (GW), and to describe their clinical manifestations and their response to treatment. METHODS: We evaluated 50 consecutive patients with GW, come to our attention from January 1987 to May 2003. 43 patients met the 1990 American College of Rheumatology (ACR) criteria for classification of GW; 7 patients the 1993 ELK criteria. RESULTS: 82% of patients presented Ear/Nose/Throat (ENT) involvement, which is the most common site of inflammation. 22% of our patients had ENT-restricted disease; in 78% of cases disease extended to other organs. Lungs were involved in 72% of cases; kidney in 36%; eye in 24%; nervous system (NS) in 14% (central NS in 10% and peripheral NS in 4%); skin in 10%; heart in 8%; testis in 4%. Arthritis was present in 10% of patients. We discuss treatment of all patients and response to therapy of those 28 whose follow-up is available. CONCLUSIONS: Involvement of airways and kidney is by far the most common in GW, though potentially any other organ or system may be affected. The total rate of other organs involvement is 70%.


Granulomatosis with Polyangiitis/complications , Adolescent , Adult , Aged , Child , Female , Granulomatosis with Polyangiitis/drug therapy , Humans , Male , Middle Aged
17.
Neurol Sci ; 26 Suppl 1: S9-14, 2005 May.
Article En | MEDLINE | ID: mdl-15883701

Rheumatological diseases can involve the central and the peripheral nervous system in many ways. Every structure-the brain, meninges, spinal cord, cranial nerves, peripheral nerves-can be affected. Early recognition of neurological abnormality can help achieving diagnosis of the underlying condition and prevent permanent sensorimotor or cognitive function loss. This review focuses on the clinical presentation of the neurological involvement in rheumatological diseases.


Nervous System Diseases/etiology , Rheumatic Diseases/physiopathology , Cognition/physiology , Connective Tissue Diseases/etiology , Humans , Rheumatic Diseases/diagnosis , Sarcoidosis/etiology , Vasculitis/etiology
18.
Arthritis Rheum ; 53(1): 100-7, 2005 Feb 15.
Article En | MEDLINE | ID: mdl-15696576

OBJECTIVE: Takayasu's arteritis (TA) is a rare vasculitis. The Italian Takayasu's Arteritis study group was established with the aim to describe a large cohort of patients. METHODS: Data were collected by means of an ad hoc form. Demographic information, clinical history, vascular findings, treatment, risk factors, and comorbidities were analyzed. RESULTS: Data of 104 patients were collected. The median delay in diagnosis was 15.5 months (range 0-325 months). Age at onset <15 years was associated with a higher probability, whereas elevated erythrocyte sedimentation rate with a lower probability, of a delay in diagnosis. The majority of patients experienced nonspecific signs and symptoms indicative of an inflammatory disease in the early phase. Among vascular involvement, stenosis was the most frequent lesion, being present in 93% of patients, followed by occlusion (57%), dilatation (16%), and aneurysm (7%). Glucocorticoids were the mainstay of treatment in our series; however, treatment with cytotoxic agents was required in about half of the patients. Fifty-two patients underwent at least 1 surgical procedure. The main indications for intervention were renal vascular hypertension, cerebral hypoperfusion, and limb claudication. CONCLUSION: As with many rare diseases, delay in diagnosis is an important issue for patients with TA. The increasing occurrence of vascular lesions along with the disease progression put to question the long-term effectiveness of contemporary treatment. These data may be helpful in increasing physicians' awareness to prevent diagnosis delay, update guidelines, and plan future research projects.


Takayasu Arteritis/diagnosis , Takayasu Arteritis/epidemiology , Adult , Age of Onset , Angiography , Blood Sedimentation , Female , Glucocorticoids/therapeutic use , Humans , Italy/epidemiology , Male , Middle Aged , Pregnancy , Pregnancy Complications/epidemiology , Risk Factors , Takayasu Arteritis/drug therapy , Takayasu Arteritis/surgery
19.
Arthritis Rheum ; 52(2): 539-47, 2005 Feb.
Article En | MEDLINE | ID: mdl-15692970

OBJECTIVE: To test the association of osteopontin (OPN) polymorphisms with systemic lupus erythematosus (SLE). METHODS: The coding 5' and 3' flanking regions of the OPN gene were scanned for polymorphisms by denaturing high-performance liquid chromatography. A case-control association study was performed in 394 Italian SLE patients and 479 matched controls. OPN serum levels were determined by enzyme-linked immunosorbent assay in 40 patients and 124 controls, and the mean levels were compared between the different OPN genotypes. RESULTS: Among the 13 detected single-nucleotide polymorphisms (SNPs), alleles -156G (frequency 0.714 versus 0.651; P = 0.006, corrected P [P(corr)] = 0.036) and +1239C (0.377 versus 0.297; P = 0.00094, P(corr) = 0.0056) were significantly increased in the SLE patients compared with the controls. The presence of the associated allele in single or double dose conferred an odds ratio (OR) of 2.35 (95% confidence interval [95% CI] 1.38-4.02) for SNP -156 and an OR of 1.57 (95% CI 1.16-2.13) for SNP +1239. These effects were independent of each other, i.e., not a consequence of linkage disequilibrium between the 2 alleles. The risk associated with a double dose of susceptibility alleles at both SNPs was 3.8-fold higher (95% CI 2.0-7.4) relative to the complete absence of susceptibility alleles. With regard to individual clinical and immunologic features, a significant association was seen between lymphadenopathy and -156 genotypes (overall P = 0.0011, P(corr) = 0.046). A significantly increased OPN serum level was detected in healthy individuals carrying +1239C (P = 0.002), which is indicative of an association between the SLE susceptibility allele and OPN levels. CONCLUSION: These data suggest the independent effect of a promoter (-156) and a 3'-untranslated region (+1239) SNP in SLE susceptibility. We can speculate that these sequence variants (or others in perfect linkage disequilibrium) create a predisposition to high production of OPN, and that this in turn may confer susceptibility to SLE.


Disease Susceptibility , Lupus Erythematosus, Systemic/genetics , Polymorphism, Single Nucleotide , Sialoglycoproteins/genetics , Female , Humans , Male , Osteopontin , Sialoglycoproteins/blood
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