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1.
Allergy ; 72(2): 177-182, 2017 Feb.
Article in English | MEDLINE | ID: mdl-27564982

ABSTRACT

BACKGROUND: Schnitzler syndrome is characterized by an urticarial rash, a monoclonal gammopathy, and clinical, histological, and biological signs of neutrophil-mediated inflammation. The aim of this study was to assess the applicability and validity of the existing diagnostic criteria in real-life patients. METHODS: This multicentric study was conducted between 2009 and 2014 in 14 hospitals in which patients with Schnitzler syndrome or controls with related disorders were followed up. We compared the sensitivities and specificities and calculated the positive and negative predictive values of the Lipsker and of the Strasbourg criteria for the patients with Schnitzler syndrome and for the controls. We included 42 patients with Schnitzler syndrome, 12 with adult-onset Still's disease, 7 with cryopyrin-associated periodic disease, 9 with Waldenström disease, and 10 with chronic spontaneous urticaria. RESULTS: All patients with Schnitzler syndrome met the Lipsker criteria. According to the Strasbourg criteria, 34 patients had definite Schnitzler syndrome, five had probable Schnitzler syndrome, and three did not meet the criteria. One control met the Lipsker criteria and had probable Schnitzler syndrome according to the Strasbourg criteria. Sensitivity and specificity of the Lipsker criteria were 100% and 97%, respectively. For the Strasbourg criteria, sensitivity for definite and probable diagnosis was 81% and 93%, respectively, with a corresponding specificity of 100% and 97%. CONCLUSION: Diagnostic criteria currently in use to diagnose Schnitzler syndrome are reliable. More investigations must be done to attest their efficiency in patients with recent-onset manifestations.


Subject(s)
Schnitzler Syndrome/diagnosis , Adolescent , Adult , Child , Child, Preschool , Female , Humans , Male , Reproducibility of Results , Sensitivity and Specificity , Severity of Illness Index , Symptom Assessment , Young Adult
2.
Lupus ; 20(8): 861-5, 2011 Jul.
Article in English | MEDLINE | ID: mdl-21546412

ABSTRACT

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by arterial and/or venous thromboses and/or pregnancy-associated morbidity. Some patients develop only obstetric complications (obstetric APS), but data on the frequency of thrombotic events during the follow-up of these patients are scarce. This study was undertaken to evaluate the rate of thrombotic events after obstetric APS diagnosis according to the 2006 revised criteria. In total, 32 obstetric APS patients were retrospectively studied, with mean follow-up of 50 ± 37 months. After delivery, aspirin was prescribed to all patients as primary thrombosis prevention. The thrombosis rate was 3.3/100 patient-years and was 4.6, 4.5 and 10/100 patient-years when we considered at least two antiphospholipid antibody positivities (among lupus anticoagulant, anticardiolipin and anti-ß2-glycoprotein-I), antinuclear antibody positivity or systemic lupus erythematosus-associated APS patients, respectively. The thrombosis rate was high after obstetric APS diagnosis, even for patients taking aspirin. Larger, prospective studies are needed to confirm this high frequency and determine the associated risk factors.


Subject(s)
Antiphospholipid Syndrome/complications , Pregnancy Complications/immunology , Thrombosis/etiology , Antibodies, Antinuclear/blood , Antibodies, Antinuclear/immunology , Antibodies, Antiphospholipid/blood , Antibodies, Antiphospholipid/immunology , Antiphospholipid Syndrome/immunology , Antiphospholipid Syndrome/physiopathology , Aspirin/therapeutic use , Female , Fibrinolytic Agents/therapeutic use , Follow-Up Studies , Humans , Pregnancy , Retrospective Studies , Risk Factors , Thrombosis/immunology , Thrombosis/prevention & control , Young Adult
3.
Rev Med Interne ; 30(7): 628-9, 2009 Jul.
Article in French | MEDLINE | ID: mdl-18930567

ABSTRACT

The association of sarcoidosis and auto-immune thyroid disease has been reported. We report a 29-year-old woman, treated for hypothyroidism caused by thyrotropin-receptor blocking antibodies, who developed a sarcoidosis (Löfgren's syndrome). Auto-immune thyroid diseases and sarcoidosis could share common pathogenic mechanisms.


Subject(s)
Autoantibodies/blood , Hypothyroidism/immunology , Receptors, Thyrotropin/immunology , Sarcoidosis/diagnosis , Sarcoidosis/immunology , Adult , Female , Humans
4.
Rev Med Interne ; 29(10): 805-7, 2008 Oct.
Article in French | MEDLINE | ID: mdl-18572278

ABSTRACT

INTRODUCTION: Bromocriptin has been associated with stroke and myocardial infarction in the postpartum period. We report on the case of a patient who developed digital ischemia while receiving this drug. EXEGESIS: Mrs D, 28 years old presented with digital ischemia occurring five days after the introduction of bromocriptin. Magnetic resonance imaging also displayed stroke in the area of the right posterior cerebellar artery. The course was favourable after discontinuation of the drug. DISCUSSION: Bromocriptin is an ergot derivative with dopaminergic agonist properties. A paradoxical vasoconstriction is rarely associated with vascular ischemic complications, including digital ischemia.


Subject(s)
Bromocriptine/adverse effects , Dopamine Agonists/adverse effects , Fingers/blood supply , Ischemia/chemically induced , Adult , Bromocriptine/administration & dosage , Depression, Postpartum/drug therapy , Dopamine Agonists/administration & dosage , Female , Humans
6.
Scand J Rheumatol ; 34(5): 399-403, 2005.
Article in English | MEDLINE | ID: mdl-16234190

ABSTRACT

Adult Still's disease (ASD) is a rare systemic disorder characterized by fever, arthralgia, cutaneous rash, and lymphadenopathy, with high polymorphonuclear leucocytosis and low glycosylated ferritinaemia. Kidney involvement has been reported rarely. We present a patient with ASD who developed haemolytic uraemic syndrome (HUS). The 42-year-old patient was admitted for unexplained fever related to ASD according to Yamaguchi's classification criteria. As Still's disease was resistant to prednisone, high-dose intravenous immunoglobulins (IV Ig) were administered. During the follow-up the patient developed acute renal failure and non-immune haemolytic anaemia with high levels of antiphospholipid antibodies (IgG anticardiolipin antibodies and anti-beta2 glycoprotein 1 antibodies). Renal biopsy disclosed thrombotic microangiopathy (TMA) with arteriolar and glomerular involvement. Treatment with steroids and intravenous IV Ig was reinitiated but renal function worsened towards end-stage renal failure. In this case, we suggest that antiphospholipid antibodies could have promoted arteriolar and glomerular TMA. HUS may be the cause of acute renal failure in Still's disease.


Subject(s)
Acute Kidney Injury/etiology , Hemolytic-Uremic Syndrome/etiology , Still's Disease, Adult-Onset/complications , Thrombosis/etiology , Acute Kidney Injury/immunology , Adult , Antibodies, Antiphospholipid/immunology , Hemolytic-Uremic Syndrome/immunology , Humans , Immunoglobulins, Intravenous , Male , Microcirculation , Renal Circulation , Steroids/therapeutic use , Still's Disease, Adult-Onset/drug therapy , Still's Disease, Adult-Onset/immunology , Thrombosis/immunology
7.
Presse Med ; 34(12): 861-2, 2005 Jul 02.
Article in French | MEDLINE | ID: mdl-16097210

ABSTRACT

INTRODUCTION: Familial forms of small-vessel vasculitis has been reported in 14 families (including this one). CASES: A father and son were both diagnosed with renal vasculitis (pauci-immune crescentic glomerulonephritis). Both had antimyeloperoxidase autoantibodies, and there was no evidence of a common environmental factor. DISCUSSION: These cases suggest the role of constitutional factors in the pathogenesis of antineutrophil cytoplasmic antibody-associated vasculitis.


Subject(s)
Kidney/blood supply , Vasculitis/genetics , Aged , Humans , Male , Middle Aged
8.
Ann Rheum Dis ; 64(6): 913-20, 2005 Jun.
Article in English | MEDLINE | ID: mdl-15550531

ABSTRACT

OBJECTIVE: To assess the tolerance and efficacy of rituximab in patients with various autoimmune diseases seen in daily rheumatological practice. METHODS: 866 rheumatology and internal medicine practitioners were contacted by e-mail to obtain the files of patients treated with rituximab for systemic autoimmune diseases. Patients with lymphoma were analysed if the evolution of the autoimmune disease could be evaluated. RESULTS: In all, 43 of 49 cases could be analysed, including 14 with rheumatoid arthritis (RA), 13 with systemic lupus erythematosus (SLE), six with primary Sjogren's syndrome (pSS), five with systemic vasculitis, and five with other autoimmune diseases. Rituximab was prescribed for lymphoma in two patients with RA and two with pSS. In the 39 other cases, rituximab was given because of the refractory character of the autoimmune disease. The mean follow up period was 8.3 months (range 2 to 26). There were 11 adverse events in 10 patients and treatment had to be discontinued in six. Efficacy was observed in 30 patients (70%): RA 11, SLE 9, pSS 5, vasculitis 2, antisynthetase syndromes 2, sarcoidosis 1. The mean decrease in corticosteroid intake was 9.5 mg/d (range 0 to 50) in responders. Seven patients experienced relapse after mean 8.1 months (5 to 15). Three patients died because of refractory autoimmune disease. CONCLUSIONS: Despite absence of marketing authorisation, rituximab is used to treat various refractory autoimmune diseases in daily rheumatological practice. This study showed good tolerance and short term clinical efficacy, with marked corticosteroid reduction in patients with SLE, pSS, vasculitis, and polymyositis.


Subject(s)
Antibodies, Monoclonal/therapeutic use , Antirheumatic Agents/therapeutic use , Autoimmune Diseases/drug therapy , Rheumatic Diseases/drug therapy , Adult , Aged , Aged, 80 and over , Antibodies, Monoclonal/adverse effects , Antibodies, Monoclonal, Murine-Derived , Antirheumatic Agents/adverse effects , Arthritis, Rheumatoid/drug therapy , Drug Administration Schedule , Drug Therapy, Combination , Female , Glucocorticoids/administration & dosage , Humans , Immunologic Factors/adverse effects , Immunologic Factors/therapeutic use , Lupus Erythematosus, Systemic/drug therapy , Male , Middle Aged , Retrospective Studies , Rituximab , Sjogren's Syndrome/drug therapy , Treatment Outcome , Vasculitis/drug therapy
9.
Clin Nephrol ; 61(6): 434-9, 2004 Jun.
Article in English | MEDLINE | ID: mdl-15224808

ABSTRACT

Tubulointerstitial nephritis is the most common renal complication in primary Sjögren's syndrome (SS). It is usually associated with symptoms of distal tubular dysfunction, type I (distal) renal tubular acidosis (RTA) and nephrogenic diabetes insipidus. Proximal tubular abnormalities are considered to be less frequent, and Fanconi's syndrome has been only exceptionally reported in patients with SS. We describe 2 patients with primary SS, characterized by xerostomia, dry eyes, extensive lymphocytic infiltrate on salivary gland biopsy, positive tests for anti-SSA/SSB antibodies and/or antinuclear antibodies, who presented in renal failure with proteinuria, microscopic hematuria and type I RTA. Further studies revealed proximal tubular dysfunction, including renal glucosuria, generalized aminoaciduria, phosphaturia, uricosuria, together with proximal (type II) RTA in 1 case. Neither of these patients had Bence Jones proteinuria or monoclonal gammopathy. Kidney biopsy showed focal proximal tubulitis, associated with proximal tubular cell atrophy and dedifferentiation, and diffuse interstitial nephritis with fibrosis. No significant glomerular or peritubular deposits of immunoglobulin light or heavy chain were observed. These findings demonstrate that diffuse, distal and proximal, tubular dysfunction may occur in patients with SS and interstitial nephritis. Lymphocytic infiltration of proximal tubular cells is probably involved in the pathogenesis of Fanconi's syndrome in SS. However, the mechanisms involved in the alteration of sodium-dependent apical transports remain to be elucidated.


Subject(s)
Nephritis, Interstitial/etiology , Sjogren's Syndrome/complications , Adult , Aged , Fatal Outcome , Female , Humans , Kidney/pathology , Male , Nephritis, Interstitial/pathology , Sjogren's Syndrome/pathology
10.
Rev Med Interne ; 25(4): 294-8, 2004 Apr.
Article in French | MEDLINE | ID: mdl-15050796

ABSTRACT

PURPOSE: Rhabdomyolysis and myositis are rare, dose-related complications of statins and fenofibrates. The outcome is favorable as a rule with rapid regression after stopping the responsible drug. Recently, various auto-immune disease with evidence of hypersensitivity to HMG-CoA reductase inhibitors or fibrates drugs have been reported. Less than ten cases of dermatomyositis and polymyositis due to cholesterol-lowering drugs (CLD) have been previously reported. Five more cases polymyositis associated with CLD are reported. METHODS: Symptoms were compatible with diagnosis of polymyositis according to Bohan and Peter and with previous reported criteria for drug-induced myopathy in all cases. None of these patients had previous other connective tissue disorders. RESULTS: Five patients (median age 68 [54-78], female N =4) with CLD treatment (statin N =4, fenofibrates N =1) have developed iatrogenic polymyositis. All of them presented both proximal muscular weakness and increased muscle enzyme levels. One patient had iatrogenic antisynthetase syndrome characterized by mechanic's hand, Raynaud's phenomenon and anti JO1 antibodies. One other had sclerodermic hand oedema. Antinuclear antibodies were positive in 4 cases and muscle biopsy revealed polymyositis infiltrate in 4 cases. CLD treatment was discontinued with partial clinical improvement in 3 cases. Clinical remission was obtained with corticosteroid (N =5) in association with immunosuppresive agents in 3 cases. CONCLUSION: Muscular symptoms in patient with CLD treatment could be the first symptom of a polymyositis revealed or increased by this treatment and must encourage physician with antinuclear antibodies screening especially in case of proximal muscular weakness and increased muscle enzyme levels.


Subject(s)
Fenofibrate/adverse effects , Hydroxymethylglutaryl-CoA Reductase Inhibitors/adverse effects , Hypolipidemic Agents/adverse effects , Polymyositis/chemically induced , Aged , Antibodies, Antinuclear/analysis , Female , Fenofibrate/therapeutic use , Humans , Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use , Hypercholesterolemia/drug therapy , Hypolipidemic Agents/therapeutic use , Male , Middle Aged
11.
Arch Mal Coeur Vaiss ; 96(9): 919-22, 2003 Sep.
Article in French | MEDLINE | ID: mdl-14571647

ABSTRACT

The cardiac effects of dermatomyositis and polymyositis are found frequently at autopsy, even though the clinical manifestations are rare. We report the observation of a patient with dermatomyositis, in whom ventricular tachycardia, and dilated hypokinetic cardiomyopathy were in the foreground of the cardiac anomalies. This rhythm disturbance responds to treatment, as opposed to the associated dilated cardiomyopathy. Electrocardiographic anomalies are frequent, notably conduction disturbances. Ventricular rhythm disturbances are of poorly defined frequency. Cardiac insufficiency is often stabilised with treatment. The other effects are much rare.


Subject(s)
Cardiomyopathies/etiology , Dermatomyositis/complications , Tachycardia, Ventricular/etiology , Aged , Cardiomyopathies/pathology , Echocardiography , Humans , Male , Tachycardia, Ventricular/drug therapy , Tachycardia, Ventricular/pathology
12.
Nephrologie ; 22(6): 301-5, 2001.
Article in French | MEDLINE | ID: mdl-11715607

ABSTRACT

Puumala hantavirus is the most common hantavirus infection in Western Europe. The causative agent, Puumala virus, is a member of the Hantavirus genus in the Bunyaviridae family. The natural hosts of hantaviruses are chronically, but asymptomatic infected rodents, which transmit the virus to human in their excretions. Puumala virus is carried by the bank vole, clethrionomys glareolus. Hemorrhagic fever with renal syndrome (HFRS) caused by Puumala virus in France or Belgium is very similar to the previously described Nephropathia epidemica in Scandinavia. In most severe cases, the disease is clinically characterized by high fever of abrupt onset, headache, loin or abdominal pains, nausea and vomiting, and occasionally acute and transient myopia. Renal involvement results in transient proteinuria and hematuria and acute renal failure. Except for interstitial hemorrhage in the outer medulla, the renal histopathologic findings are unspecific and include prominent changes in the interstitium with interstitial oedema and inflammatory infiltrates. Thrombocytopenia, mild elevation of liver enzymes, and leukocytosis are typical laboratory findings. Spontaneous complete recovery is the rule. Laboratory diagnosis is primarily based on serology such as indirect immunofluorescence or capture enzyme--linked immunosorbent assays which detect IgM antibodies and an increased level of IgG antibodies against Puumala virus. Viral antigen may be demonstrated in the cytoplasm of renal tubular epithelial cells.


Subject(s)
Hemorrhagic Fever with Renal Syndrome/virology , Puumala virus/pathogenicity , Acute Kidney Injury/etiology , Animals , Antibodies, Viral/blood , Asia/epidemiology , Belgium/epidemiology , Disease Progression , Disease Vectors , France/epidemiology , Orthohantavirus/classification , Orthohantavirus/isolation & purification , Orthohantavirus/pathogenicity , Hemorrhagic Fever with Renal Syndrome/complications , Hemorrhagic Fever with Renal Syndrome/diagnosis , Hemorrhagic Fever with Renal Syndrome/epidemiology , Hemorrhagic Fever with Renal Syndrome/immunology , Hemorrhagic Fever with Renal Syndrome/transmission , Humans , Immunoglobulin G/blood , Immunoglobulin M/blood , Kidney Tubules/virology , Pneumonia, Viral/etiology , Puumala virus/immunology , Puumala virus/isolation & purification , Rodentia/virology , Scandinavian and Nordic Countries/epidemiology , Seroepidemiologic Studies , Zoonoses
13.
Am J Med ; 106(5): 527-33, 1999 May.
Article in English | MEDLINE | ID: mdl-10335724

ABSTRACT

PURPOSE: To assess the value of serial determinations of antineutrophil cytoplasmic autoantibodies (ANCA) for monitoring disease activity in patients with systemic vasculitis. PATIENTS AND METHODS: Forty-three patients with histologically proven vasculitis (21 with Wegener's granulomatosis, 17 with microscopic polyangiitis, and 5 with renal-limited vasculitis) were studied for a median follow-up of 22 months. Disease activity was prospectively assessed and quantified by the Birmingham Vasculitis Activity Score. A total of 347 sera were analyzed for ANCA determination. RESULTS: Relapses occurred in 23 (54%) of 43 patients. Diagnostic category (Wegener's granulomatosis vs micropolyangiitis and renal-limited vasculitis), severity of initial symptoms (mean vasculitis activity score, mean number of organs involved), and ANCA pattern [cytoplasmic-ANCA (c-ANCA) vs perinuclear-ANCA (p-ANCA)] did not significantly differ between relapsers and nonrelapsers. Lung involvement was more frequent at onset among relapsers [16 of 23 (70%) vs 6 of 20 (30%); P = 0.02]. Relapses were slightly, but not significantly, more frequent in patients with Wegener's granulomatosis or a c-ANCA pattern. The percentage of relapsers was greater in patients with persistently positive ANCA than in patients with negative or decreasing ANCA titers (86% vs 20%, P = 0.0001). However, the predictive value of an increase in ANCA titers for the occurrence of a subsequent relapse was only 28% (4 of 14) for c-ANCA, 12% (2 of 17) for anti-proteinase 3-ANCA, and 43% (6 of 14) for anti-myeloperoxidase-ANCA. An increase in ANCA occurred before or during relapse in 33% (10 of 30) of cases for c-ANCA/anti-proteinase 3 antibodies, and 73% (11 of 15) of cases for anti-myeloperoxidase antibodies. CONCLUSION: The persistence of ANCA positivity is strongly associated with relapses. However, an increase in ANCA titers has a poor value for the early prediction of a subsequent relapse and should not be used as a sole parameter for therapeutic intervention. In addition, our results suggest that serial anti-myeloperoxidase determination may be useful as a prognostic marker in patients who are p-ANCA positive.


Subject(s)
Antibodies, Antineutrophil Cytoplasmic/blood , Vasculitis/immunology , Aged , Arteritis/immunology , Endopeptidases/immunology , Female , Granulomatosis with Polyangiitis/immunology , Humans , Male , Middle Aged , Peroxidase/immunology , Predictive Value of Tests , Recurrence , Vasculitis/enzymology
14.
Presse Med ; 28(2): 67-70, 1999 Jan 16.
Article in French | MEDLINE | ID: mdl-9989295

ABSTRACT

BACKGROUND: Side-effects, including autoimmune disorders, are frequent with D-penicillamine therapy. Proteinuria is observed in 10% of the patients, often secondary to extramembranous glomerulopathy. Necrotizing extracapillary glomerulonephritis is however exceptional. CASE REPORTS: Two patients with systemic sclerodermia were treated with D-penicillamine for 7 and 14 years. Both developed necrotizing extracapillary glomerulonephritis with anti-myeloperoxidase antibodies (anti-MPO), associated with hemorrhagic alveolitis in one case. Partial regression of the renal failure was obtained after withdrawal of D-penicillamine and combination treatment with prednisone and cyclophosphamide. DISCUSSION: Extracapillary glomerulonephritis or a lung-kidney syndrome are frequently associated with anti-MPO antineutrophil cytoplasm antibodies (ANCA). In systemic sclerodermia, the presence of anti-MPO appears to define a group of patients at risk of pauci-immune extracapillary glomerulonephritis or a lung-kidney syndrome. In addition, the presence of ANCA in patients with renal failure would suggest extracapillary glomerulonephritis rather than sclerodermic microangiopathy. Development of extracapillary glomerulonephritis with anti-MPO in patients who are taking D-penicillamine suggests that inductor mechanisms other than D-penicillamine are involved in the pathogenesis of these glomerulopathies.


Subject(s)
Glomerulonephritis/immunology , Penicillamine/therapeutic use , Raynaud Disease/complications , Scleroderma, Systemic/complications , Aged , Antibodies, Antineutrophil Cytoplasmic/immunology , Autoimmune Diseases/chemically induced , Female , Glomerulonephritis/chemically induced , Humans , Middle Aged , Penicillamine/adverse effects , Proteinuria/chemically induced , Raynaud Disease/immunology , Scleroderma, Systemic/drug therapy , Scleroderma, Systemic/immunology , Sex Factors
15.
Rev Med Interne ; 20(12): 1088-92, 1999 Dec.
Article in French | MEDLINE | ID: mdl-10635070

ABSTRACT

PURPOSE: The present study was aimed at assessing the influence of age on clinical and biological features of systemic sclerosis. METHODS: This retrospective study included 151 consecutive patients with systemic sclerosis. The median age at diagnosis was 50.0 years (range: 10-84 years). Patients were divided into two groups according to their age (lower than 50.0 years of age: 73 patients, equal to or above 50 years of age: 78 patients). The following features were compared between the two groups: gender, disease duration, extent of skin sclerosis, Crest syndrome, lung fibrosis, secondary Sjögren's syndrome, antinuclear, anticentromere, and anti-Scl70 antibodies. RESULTS: The disease duration was significantly higher in patients over 50 years of age (7.1 +/- 6.8 years vs 5.5 +/- 5.0 years, P < 0.05). Crest syndrome, secondary Sjögren's syndrome and anticentromere antibodies were significantly more common in patients over 50 years of age (17/73 vs 30/78, P < 10(-2); 9/73 vs 20/78, P < 10(-2), and 19/73 vs 31/78, P < 0.05; respectively). Anti-Scl70 antibodies were significantly more common in patients under 50 years of age (17/73 vs 10/78, P < 10(-2)). No significant difference was found in regard to the other features. CONCLUSION: The clinical and biological patterns of systemic sclerosis are different according to the age at disease onset. Crest syndrome including anticentromere antibodies and Sjögren's syndrome is more common in elderly patients, while anti- Scl-70 antibodies are more common in younger patients. This suggests the involvement of various mechanisms in the pathogenesis of systemic sclerosis, and that these mechanisms may depend on the age.


Subject(s)
CREST Syndrome/etiology , Scleroderma, Systemic/pathology , Adolescent , Adult , Age Factors , Aged , Aged, 80 and over , CREST Syndrome/pathology , Centromere/genetics , Centromere/immunology , Child , Female , Humans , Male , Middle Aged , Pulmonary Fibrosis/etiology , Pulmonary Fibrosis/pathology , Retrospective Studies , Scleroderma, Systemic/complications , Scleroderma, Systemic/physiopathology , Severity of Illness Index , Sjogren's Syndrome/etiology , Sjogren's Syndrome/pathology
16.
Rev Med Interne ; 18(7): 528-32, 1997 Jul.
Article in French | MEDLINE | ID: mdl-9255370

ABSTRACT

Several studies have suggested an increased risk of cancer in patients with systemic sclerosis, but the potential risk factors for these cancers remain unknown. The aim of this study was to identify, among patients with systemic sclerosis, factors associated with the development of cancer, with attention to clinical (age, sex, cutaneous sclerosis), immunological (antinuclear antibodies, anticentromere antibodies, anti-Scl-70 antibodies) and histological (pulmonary fibrosis) features. We retrospectively studied 123 patients with systemic sclerosis. The median follow-up period was 4 years. Fourteen cases of cancer (11.3%) were found (lung n = 3, breast n = 2, ovarian n = 2, skin n = 1, thyroid n = 1, rectum n = 1, uterine cervix n = 1, larynx n = 1, pancreas n = 1, myelodysplasia n = 1). The characteristics of systemic sclerosis were similar in patients with and patients without cancer. Yet, the three cases of lung cancers occurred in association with CREST syndromes and anticentromere antibodies.


Subject(s)
Neoplasms/etiology , Scleroderma, Systemic/complications , Adolescent , Adult , Aged , Aged, 80 and over , Antibodies, Antinuclear/analysis , CREST Syndrome/complications , Female , Humans , Male , Middle Aged , Neoplasms/physiopathology , Predictive Value of Tests , Retrospective Studies , Risk Factors , Scleroderma, Systemic/immunology , Scleroderma, Systemic/physiopathology
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