Luisa Praderio - Academia.edu (original) (raw)

Papers by Luisa Praderio

Archives of internal medicine, Feb 1, 1987

To the Editor. —Pseudoxanthoma elasticum (PE) is a rare, genetically determined disease with prog... more To the Editor. —Pseudoxanthoma elasticum (PE) is a rare, genetically determined disease with progressive cutaneous, ocular, and arterial lesions. 1 Autoimmune thyroiditis but, to our knowledge, no other autoimmune disease, has been sporadically observed in patients with PE. 2 We report a case of rheumatoid arthritis in a patient who was found to have PE. Report of a Case. —A 43-year-old woman whose parents were consanguineous suffered from a symmetrical and deforming polyarthritis, mainly involving her hands and wrists, since she was 21 years of age. In 1983, a diagnosis of seronegative definite rheumatoid arthritis was made, according to the criteria of the American Rheumatism Association. Subsequently, the patient's arthritis worsened, and she was admitted to our clinic in October 1985 because of severe involvement of her left hip. Physical examination revealed typical features of active rheumatoid arthritis. Laboratory studies disclosed the following Top, Prominent juxta-articular osteoporosis and extensive erosive

Autoimmunity Reviews, Apr 1, 2015

Large-vessel GCA Claudication FDG-PET 20 Objective: Giant cell arteritis (GCA) is a systemic vasc... more Large-vessel GCA Claudication FDG-PET 20 Objective: Giant cell arteritis (GCA) is a systemic vasculitis typically affecting temporal arteries. In at least 15% of 21 cases, GCA also features inflammation of the aorta and its primary branches. Large-vessel inflammation restricted 22 to proximal limb arteries in the absence of temporal and aortic involvement (Limb Restricted, LR) is rare and not 23 well described in literature. Hence, we aim to characterize this neglected clinical entity. 24 Methods: We describe a series of three cases of LR-GCA. All patients were older than 50 years, had increased 25 erythrocyte sedimentation rate (ESR), normal cholesterol and triglycerides serum levels, negative temporal 26 artery biopsy, suggestive F-18 fluorodeoxyglucose positron emission tomography (FDG-PET) findings, and 27 responded to immunosuppressive therapy. We also reviewed all published cases of LR-GCA (76 cases), for a 28 total of 79 patients. 29 Results: Limb claudication was reported in 87% of the patients, and cranial symptoms and polymyalgia 30 rheumatica in 20%. Constitutional symptoms were never reported. Median ESR levels were 66.5 mm/1 h. 31 Upper and lower limb arteries were involved in 86% and 9% of the patients respectively, and the remaining 5% 32 had simultaneous upper and lower limb vessel involvement. Conventional angiography was performed in 63% 33 of the cases, color-doppler ultrasound in 20%, FDG-PET in 14%, and computed tomography angiography in 3%. 34 Conclusion: If temporal biopsy and aortic imaging are negative for GCA in patients older than 50 years, with 35 bilateral limb claudication, elevated ESR, and suggestive vascular radiological findings, LR-GCA should be 36 suspected. Upper limb arteries are more frequently involved. Since constitutional symptoms are typically absent 37 in LR-GCA, differential diagnosis with atherosclerotic plaques may be challenging.

The Journal of Rheumatology, Oct 1, 2013

Clinical Infectious Diseases, Nov 1, 1998

Arthritis & Rheumatism, 2001

Objective. To verify whether the prototypical long pentraxin PTX3 represents an indicator of the ... more Objective. To verify whether the prototypical long pentraxin PTX3 represents an indicator of the activity of small-vessel vasculitis. Methods. Concentrations of PTX3, a pentraxin induced in endothelium by cytokines, were measured by enzyme-linked immunosorbent assay in the sera of 43 patients with Churg-Strauss syndrome, Wegener's granulomatosis, and microscopic polyangiitis. PTX3 was also measured in the sera of 28 patients with systemic lupus erythematosus (SLE), 22 with rheumatoid arthritis, and 16 with CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias). Serum concentrations of C-reactive protein (CRP) were measured by immunoturbidimetry. The cells involved in PTX3 production in vivo were identified in skin biopsy samples. Results. Patients with active vasculitis had significantly higher concentrations of PTX3 than did those with quiescent disease (P < 0.001). PTX3 levels in the latter group were similar to those in healthy controls. PTX3 levels were higher in patients with untreated vasculitis and lower in patients who underwent immunosuppressive treatments (P < 0.005). In contrast, patients with active SLE had negligible levels of the pentraxin. PTX3 levels did not correlate with CRP levels in vasculitis patients. Endothelial cells produced PTX3 in active skin lesions. Conclusion. PTX3 represents a novel acute-phase reactant produced at sites of active vasculitis. Small-vessel vasculitis encompasses a group of disorders characterized by inflammatory damage within or through the vessel wall, accompanied by widespread systemic inflammation. In most cases, inflammation is due to immunopathogenic mechanisms (1-3) and primary proinflammatory cytokines. Tumor necrosis factor ␣ (TNF␣) and interleukin-1␤ (IL-1␤) in particular contribute to the inflammatory lesions (4), initiating or promoting the activation of endothelial cell and circulating leukocytes. These events in turn impinge on the permeability barrier and the homeostatic function of the vascular endothelium (5), promoting the firm adhesion and the transmigration of leukocytes and the eventual impairment of blood flow and of vessel and tissue integrity. Accordingly, levels of proinflammatory cytokines and their antagonists are elevated in the blood of patients with systemic vasculitis (6,7). Most patients experience active or quiescent phases during the course of vasculitis. Changes in the erythrocyte sedimentation rate (ESR) and blood concentrations of the short pentraxin C-reactive protein (CRP) are used to monitor the activity of vasculitis. Furthermore, elevations in the ESR and CRP levels

Journal of hematological malignancies, Sep 4, 2012

We describe the case of a 19-year-old boy, who presented with diffuse lymph node enlargements and... more We describe the case of a 19-year-old boy, who presented with diffuse lymph node enlargements and prominent involvement of his parotid glands. After a four-month history of unsuccessful diagnostic investigations, he was finally diagnosed with Kikuchi-Fujimoto's disease (KFD), a rare histiocytosis of unknown aetiology affecting lymph-nodes throughout the body. The diagnosis of KFD can only be made by histopathologic characterization of affected lymph-nodes, while, according to literature, clinical and imaging features can only suggest the presence of such particular disease. Furthermore, as happened in the case of our patient, KFD tends to be hardly distinguishable from lymphoid malignancies and to overlap with other systemic immune-mediated diseases, such as adult onset Still's disease and systemic lupus erythematosus. The significant involvement of intra-parotid lymph-nodes in our patient's case further extended the diagnostic spectrum to include Sjögren's syndrome.

The Journal of Allergy and Clinical Immunology, Oct 1, 2011

Vasculitis is a clinicopathologic process characterized by inflammation and damage to blood vesse... more Vasculitis is a clinicopathologic process characterized by inflammation and damage to blood vessels. A broad and heterogenous group of syndromes may result from this process, because any type, size, and location of blood vessel may be involved. The cause of these conditions remains unclear, but an autoimmune inflammatory process, characterized by involvement of both neutrophils and endothelial cells, seems to play an important role. In 1951, Churg and Strauss described a clinical syndrome of severe asthma, hypereosinophilia with eosinophilic infiltrates, eosinophilic vasculitis, and granulomata in various organs. Asthma may precede this vasculitis by many years. We report a case of antineutrophil cytoplasmic antibody-positive, pauci-immune, crescentic, necrotizing glomerulonephritis with peripheral and interstitial eosinophilia but without asthma. This is very unusual in Churg-Strauss syndrome.

Rheumatology, 1989

We describe a patient with Berger&amp;amp;amp;#39;s disease and polyarteritis nodosa. Thi... more We describe a patient with Berger&amp;amp;amp;#39;s disease and polyarteritis nodosa. This association has not been described previously in the literature. A causal relationship between the two diseases is suggested.

... Merlini'. Biotechnology Research Laboratory', IRCCS Policlinico San Matteo, Pavia; ... more ... Merlini'. Biotechnology Research Laboratory', IRCCS Policlinico San Matteo, Pavia; Institute of Medical Pathology2, University of Sassari, Sassari: Division of Internal Medicine3. IRCCS Ospedale San Raffaele, Milano, Italy. ...

PubMed, Jul 25, 2006

Meningeal involvement is a rare occurrence in Wegener's Granulomatosis (WG). A Medline search unc... more 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.

Annals of the Rheumatic Diseases, Jun 1, 2015

Background IgG4-related disease (IgG4-RD) is a rare fibro-inflammatory condition characterized by... more Background IgG4-related disease (IgG4-RD) is a rare fibro-inflammatory condition characterized by expansive lesions, serum IgG4 elevation in the majority of cases, and a prompt response to glucocorticoids (GCs) [1]. The diagnosis of IgG4-RD might be challenging because clinical manifestations are largely non-specific and overlap with other inflammatory and neoplastic conditions. The current gold standard for the diagnosis of IgG4-RD is the identification of characteristic histological features that are strikingly similar across the full range of organs affected. These features include a dense tissue fibrosis with a storiform pattern, a diffuse lymphoplasmacytic infiltrate with abundant IgG4-positive plasma cells, mild to moderate eosinophilia, and obliterative phlebitis [2]. GCs represent the first-line treatment for induction of remission, and typically lead to dramatic clinical responses in the majority of cases with both pancreatic and extra-pancreatic disease [3]. Objectives To describe the clinical features, treatment response and follow-up of a large cohort of Italian patients with IgG4-related disease (IgG4-RD) referred to a single tertiary care centre. Methods 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 (14 females, 27 males) were included in this study, 20 with “definite” IgG4-RD, and 21 with “possible” IgG4-RD. The mean age at disease onset was 62 years. The mean follow-up was 34 months (range 6-83 months). A history of atopy was present in 30% of patients. Pancreas, retroperitoneum, and major salivary glands were the most frequently involved organs. Clinical characteristics of the patients' cohort at IgG4-RD onset are summerized in table 1. Serum IgG4 were elevated in 68% of cases. Thirty-six patients were initially treated with glucocorticoids for inducing remission. IgG4-RD RI decreased from a mean of 7.8 at baseline to 2.9 after 1 month of therapy. Relapse occurred in 19/36 patients (53%) and required additional immunosuppressive drugs to maintain long-term remission. No further relapses were observed. A single case of orbital pseudotumor 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. References Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012;366:539–51. Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25(9):1181-92. Kamisawa T, Okazaki K, Kawa S, et al. Japanese consensus guidelines for management of autoimmune pancreatitis. III. Treatment and prognosis of AIP. J Gastroenterol 2010;45:471-7. Disclosure of Interest None declared

Objectives: Two evaluate the rate of different organs involvement in 50 patients with Wegener's G... more Objectives: Two 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%.

Seminars in Arthritis and Rheumatism, Dec 1, 2016

To describe the clinical, pathological, serological, and radiological characteristics of juxta-ve... more To describe the clinical, pathological, serological, and radiological characteristics of juxta-vertebral masses occurring in patients with Granulomatosis with polyangiitis (GPA). Methods. We analyzed the clinical records of patients with juxta-vertebral lesions from our GPA study cohort and reviewed the English literature for other cases of GPA with juxta-vertebral localization. Results. Out of 74 patients in our GPA study cohort, six (8%) had juxta-vertebral lesions. We found ten cases of juxta-vertebral GPA described in the English literature. Overall, juxta-vertebral lesions were detected at GPA onset in 11/16 (69%) patients, and preferentially occurred on the right side of the spine (12/15 patients, 80%). Fifteen patients (94%) with juxta-vertebral lesions had systemic GPA. Juxta-vertebral lesions were associated with back pain at GPA onset in 8/16 (50%) patients. In all of them juxta-vertebral lesions resolved or improved after treatment. Conclusions. Preference for the right-anterior side of the spine, increased 18 FDG uptake on PET scan, low or absent invasiveness of the surrounding tissues, and occurrence in the context of systemic disease were the main features of juxta-vertebral GPA. Symptomatic lesions showed a better response to immunosuppressive therapies.

Journal of Clinical Gastroenterology, Mar 1, 2000

This is the first reported case of a young patient with ulcerative colitis who developed acute Bu... more This is the first reported case of a young patient with ulcerative colitis who developed acute Budd-Chiari syndrome and was found positive for anticardiolipin antibodies. Although an association between ulcerative colitis and Budd-Chiari syndrome, and between the latter and antiphospholipid antibodies, has been shown in the literature, the coexistence of these three conditions has never been reported. The patient we describe did poorly and was considered as a candidate for liver transplantation in spite of prompt heparin therapy, probably because of the presence of multiple risk factors.

Scandinavian Journal of Rheumatology, Sep 23, 2015

Leukemia & Lymphoma, 2002

PubMed, Mar 1, 1993

Nervous system involvement is rare in progressive systemic sclerosis (PSS). We present a clinical... more Nervous system involvement is rare in progressive systemic sclerosis (PSS). We present a clinical pathological and immunological study of two patients with peripheral sensory motor neuropathy and PSS. In both, the sural nerve biopsies showed axonal degeneration with increased endoneurial connective tissue. There were also clusters of myelinated fibres indicating axonal regeneration. Only mild microangiopathic changes were evident in the endo, peri and epineurial vessels. By Western immunoblots, patients' sera contained a band of reactivity to a protein from peripheral nerve identified as collagen type I. Primary involvement of the peripheral nerves during PSS is very unusual. Abnormal production of collagen tissue and presence of microvascular disease are considered to be two possible causes of neuropathy. We think that our results suggest the important role of the connective tissue proliferation in the pathogenesis of PSS neuropathy.

Journal of Clinical Microbiology, Dec 1, 2000

We report the case of a 69-year-old man with acute pulmonary echinococcosis. A computed tomograph... more We report the case of a 69-year-old man with acute pulmonary echinococcosis. A computed tomographic scan of the thorax revealed the presence of multiple nodules in both lungs, and laboratory tests showed eosinophilia and the presence of antibodies against Echinococcus granulosus. Therapy with albendazole led to resolution of the pulmonary nodules and a normalization of the white cell count. To our knowledge this is the first described case of acute echinococcosis, as the diagnosis of this disease is usually delayed to chronic phases. Therefore, finding unexplained eosinophilia, especially in association with pulmonary nodules, should lead one to suspect acute hydatid disease.

Internal Medicine, 2015

Type II and type III cryoglobulinemic vasculitis (CV) are characterized by a deranged immune func... more Type II and type III cryoglobulinemic vasculitis (CV) are characterized by a deranged immune function due to concomitant chronic infections or rheumatic disorders. Conversely, type I CV is caused by plasma cell dyscrasia. Bortezomib is a proteasome inhibitor that is largely employed as a first-line treatment for multiple myeloma. The use of bortezomib in cases of monoclonal gammopathy of undetermined significance (MGUS)-related refractory type I CV has been reported in only four patients. In the current report, we discuss the efficacy of bortezomib treatment in a patient with type I CV, with a focus on the suitability and early application of this drug.