ABSTRACT
BACKGROUND: The aim of this study was to describe special features of patients with systemic sclerosis (SSc) diagnosed after the age of 70. PATIENTS AND METHODS: This is a retrospective study of patients aged above 70 years at the time of diagnosis of SSc and followed at an internal medicine unit between 2000 and 2015. Co-morbidities and clinical characteristics were analyzed, as well as survival at 1, 2 and 3 years. RESULTS: Of 246 patients, 27 (11%) were included (89% women, 96% Caucasians, age 78.3±4.5 years). Synchronous cancer was noted in 3 patients. SSc was mostly limited cutaneous only (24/27), with telangiectasia (63%), gastroesophageal reflux (59%) and digital ulcers (22%), and was associated with anti-centromere antibody (69%). Interstitial lung disease was not frequent (29%). Pulmonary arterial hypertension (PAH) was suspected at diagnosis of SSc in 14 cases (52%), but only 5 patients had undergone heart catheterization, with severe PAH in 3 cases. Survival at 1 and 3 years was 85.2% and 66.7%, and was worse in the case of suspected PAH, at 78.6% and 57.1% respectively. CONCLUSION: Cases of SSc diagnosed after 70 years are mostly limited cutaneous forms. Suspicion of PAH is frequent, and PAH may be the main initial sign of the disease for patients at this age. There may be association with synchronous cancer. Survival is poor.
Subject(s)
Internal Medicine , Late Onset Disorders/diagnosis , Scleroderma, Systemic/diagnosis , Skin Neoplasms/diagnosis , Aged , Aged, 80 and over , Female , Follow-Up Studies , France/epidemiology , Gastroesophageal Reflux/complications , Humans , Late Onset Disorders/mortality , Lung Diseases, Interstitial/complications , Male , Retrospective Studies , Risk Factors , Scleroderma, Systemic/complications , Scleroderma, Systemic/mortality , Skin Neoplasms/complications , Skin Neoplasms/mortality , Skin Ulcer/complications , Telangiectasis/complicationsABSTRACT
About ten to fifteen percent of the French population suffer from Raynaud's phenomenon. Most of the time, it is considered as primary Raynaud's phenomenon, without underlying disease. The aim of this expert consensus from the "microcirculation group" for the French Society of Vascular Medicine and the French Society for Microcirculation, was to define clinical guidelines in patients consulting for Raynaud's phenomenon. The recommended minimal screening includes clinical examination, nailfold capillaroscopy and antinuclear antibodies. In particular, the aim of this screening is to identify patients with a significant risk for scleroderma, who would need a careful follow up.
Subject(s)
Raynaud Disease/diagnosis , Antibodies, Antinuclear/blood , Connective Tissue Diseases/complications , Disease Progression , Fingers/blood supply , France/epidemiology , Humans , Laser-Doppler Flowmetry , Microcirculation , Microscopic Angioscopy , Occupational Diseases/diagnosis , Physical Examination/methods , Raynaud Disease/epidemiology , Raynaud Disease/etiology , Raynaud Disease/pathology , Raynaud Disease/physiopathology , Risk Factors , Scleroderma, Systemic/complications , Scleroderma, Systemic/diagnosis , Scleroderma, Systemic/immunologyABSTRACT
INTRODUCTION: The face is frequently involved in systemic sclerosis. The main stomatologic manifestations include limited mouth opening, xerostomia, skin atrophy, trigeminal neuralgia. The objective of this study was to describe oral and facial manifestations observed in scleroderma patients from our cohort. METHODS: Between March and October 2006, a stomatologic consultation was included in the follow-up of scleroderma patients seen during consultation or daily hospital in internal medicine or dermatology units. Demographic, clinical and biological data were collected. Stomatologic examination comprised measure of the mouth opening, sugar's and Schirmer's tests, orthopantomogram analysis, and evaluation of the repercussion of symptoms on quality of life using a visual analogical scale (VAS between 0 and 10). RESULTS: This study included 30 patients (women 87 %, mean age 58.6 + or - 13.6 years). Mean duration of systemic sclerosis (n=20 limited cutaneous form, n=10 diffuse form) was eight years. Stomatologic manifestations were: skin atrophy (n=28), peribuccal rhagades (n=25), telangiectasia (n=21), decreased mouth opening (n=20), xerostomia (n=20), xerophtalmia (n=16), periodontal ligament space widening (n=10), bone resorptions (n=2), trigeminal neuralgia (n=1). Xerostomia was considered more discomforting (mean VAS=3.8) than decreased mouth opening (mean VAS=2.6). Xerostomia was the second more discomforting sign of scleroderma and was significantly associated to the limited cutaneous form (p=0.045) and to anticentromeres antibodies expression (p=0.002). Decreased mouth opening was correlated to oesophageal involvement (p=0.025). CONCLUSION: Oral and facial manifestations are frequently observed in scleroderma patients. These manifestations lead to major functional discomfort, mainly due to decreased mouth opening that seems to be frequently associated to oesophageal involvement. Xerostomia is also frequent and is commonly observed in anticentromere antibodies positive cutaneous limited forms of systemic sclerosis. Evolution of radiographic abnormalities like periodontal ligament space widening (33 % of cases), or osteolytic lesions (7 %) is poorly known.
Subject(s)
Face , Mouth Diseases/diagnosis , Scleroderma, Systemic/diagnosis , Adult , Aged , Aged, 80 and over , Alveolar Bone Loss/diagnosis , Cohort Studies , Female , Follow-Up Studies , Humans , Male , Middle Aged , Mouth/physiopathology , Movement , Periodontal Diseases/diagnosis , Periodontal Ligament/pathology , Prospective Studies , Quality of Life , Radiography, Panoramic , Scleroderma, Diffuse/diagnosis , Scleroderma, Limited/diagnosis , Telangiectasis/diagnosis , Trigeminal Neuralgia/diagnosis , Xerophthalmia/diagnosis , Xerostomia/diagnosisABSTRACT
Antiangiogenic agents are an innovative oral chemotherapy prescribed in metastatic renal cancer and gastrointestinal stromal tumors (GIST). These molecules have several side effects. A woman, with moderate hypertension and severe Thevenard's ulceromutilating acropathy, presented renal cancer with lung metastasis. She was treated by antiangiogenic therapy (sunitinib). Under this treatment, she presented some large, extensive, severe and necrotizing ulcerations of both hands and feet, exacerbated with a sepsis. Sunitinib was stopped and antibiotics were combined with surgical trimming leading to clinical remission and complete healing. Sunitinib inhibits both tumor angiogenesis and tumor cell proliferation, but also the preexisting microcirculation. In our case, severe neuropathy caused neurovascular dysregulation which, together with hypertensive microangiopathy, led to a severe hand-foot skin reaction. This microangiopathy worsened under anti-VEGF therapy. The clinical severity was linked to the severity of the neuropathy. To avoid having serious cutaneous consequences, neuropathy and microangiopathy have to be diagnosed before introducing antiangiogenic therapy.
Subject(s)
Angiogenesis Inhibitors/adverse effects , Hereditary Sensory and Autonomic Neuropathies/complications , Skin Ulcer/chemically induced , Skin Ulcer/pathology , Anti-Bacterial Agents/therapeutic use , Antineoplastic Agents/adverse effects , Female , Foot , Hand , Humans , Indoles/adverse effects , Kidney Neoplasms/drug therapy , Lung Neoplasms/drug therapy , Lung Neoplasms/secondary , Middle Aged , Necrosis , Pyrroles/adverse effects , Skin Ulcer/surgery , SunitinibABSTRACT
INTRODUCTION: The face is frequently involved in systemic sclerosis. The main stomatologic manifestations include limited mouth opening, xerostomia, skin atrophy, trigeminal neuralgia. The objective of this study was to describe oral and facial manifestations observed in scleroderma patients from our cohort. METHODS: Between March and October 2006, a stomatologic consultation was included in the follow-up of scleroderma patients seen during consultation or daily hospital in internal medicine or dermatology units. Demographic, clinical and biological data were collected. Stomatologic examination comprised measure of the mouth opening, sugar's and Schirmer's tests, orthopantomogram analysis, and evaluation of the repercussion of symptoms on quality of life using a visual analogical scale (VAS between 0 and 10). RESULTS: This study included 30 patients (women 87%, mean age 58.6+/-13.6 years). Mean duration of systemic sclerosis (n=20 limited cutaneous form, n=10 diffuse form) was eight years. Stomatologic manifestations were: skin atrophy (n=28), peribuccal rhagades (n=25), telangiectasia (n=21), decreased mouth opening (n=20), xerostomia (n=20), xerophtalmia (n=16), periodontal ligament space widening (n=10), bone resorptions (n=2), trigeminal neuralgia (n=1). Xerostomia was considered more discomforting (mean VAS=3.8) than decreased mouth opening (mean VAS=2.6). Xerostomia was the second more discomforting sign of scleroderma and was significantly associated to the limited cutaneous form (p=0.045) and to anticentromeres antibodies expression (p=0.002). Decreased mouth opening was correlated to oesophageal involvement (p=0.025). CONCLUSION: Oral and facial manifestations are frequently observed in scleroderma patients. These manifestations lead to major functional discomfort, mainly due to decreased mouth opening that seems to be frequently associated to oesophageal involvement. Xerostomia is also frequent and is commonly observed in anticentromere antibodies positive cutaneous limited forms of systemic sclerosis. Evolution of radiographic abnormalities like periodontal ligament space widening (33% of cases), or osteolytic lesions (7%) is poorly known.
Subject(s)
Mouth Diseases/diagnosis , Scleroderma, Systemic/diagnosis , Sjogren's Syndrome/diagnosis , Xerostomia/diagnosis , Adult , Aged , Aged, 80 and over , Cohort Studies , Data Interpretation, Statistical , Female , Humans , Male , Middle Aged , Oral Medicine , Prospective Studies , Radiography, Panoramic , Scleroderma, Limited/diagnosis , Surveys and QuestionnairesABSTRACT
AIM: To describe the clinical features and etiologies of upper limb venous thrombosis (ULVT). METHODS: All patients with a clinically suspected ULVT, were included retrospectively from January to December 2016. Diagnosis of ULVT was based on doppler-ultrasonography. Clinical features, topography and symptomatic pulmonary embolism (PE) were analyzed. The sensitivity (Se), specificity (Sp), positive predictive value (PPV) and negative value (NPV) of clinical symptoms leading to ULVT suspicion were estimated by comparing patients with and without ULVT. RESULTS: Among 488 patients with a suspected ULVT, 160 were diagnosed with ULVT, including, 80 with deep venous thrombosis (DVT) and 80 with superficial venous thrombosis (SVT). Symptomatic PE was found in 2.5 % of cases (n=4). None of the clinical symptoms of ULVT had a sensitivity greater than 40 %. For DVT, presence of superior vena cava syndrome had a 100 % PPV, 71.6 % NPV and 100 % Sp. For SVT, the presence of an cord-like induration had a 85.7 % PPV, 75.3 % NPV and 98.4 % Sp. An endovenous device was present in 87.5 % of DVT and 97.5 % of SVT cases. Malignant hemopathy was found in 43.8 % and 31.3 % of cases of DVT and SVT, respectively. Sepsis and solid neoplasia were present in 25 % and 15 % of cases of ULVT, respectively. Peripherally inserted central catheter or implantable sites were present in 40 % and 17.5 % of DVT patients. No solid neoplasia, hematological malignancy or thrombophilia were diagnosed in patients with ULVT. CONCLUSION: An endovenous device was involved in 92.5 % of cases of ULVT. The prevalence of symptomatic PE was low. Hematological malignancies, sepsis and neoplasia were the most common conditions present in patients with ULVT.
Subject(s)
Pulmonary Embolism/epidemiology , Upper Extremity Deep Vein Thrombosis/diagnosis , Adult , Aged , Echocardiography, Doppler , Female , France/epidemiology , Humans , Male , Middle Aged , Prevalence , Pulmonary Embolism/etiology , Retrospective Studies , Risk Factors , Sensitivity and Specificity , Upper Extremity/blood supply , Upper Extremity Deep Vein Thrombosis/epidemiology , Upper Extremity Deep Vein Thrombosis/etiologyABSTRACT
PURPOSE: The increased risk of thromboembolism in acute medical illnesses (AMI) is difficult to assess because of the diversity of medical conditions. The first part of this review of the literature was dedicated to methods of risk analysis based on our current pathophysiological knowledge. This second part describes more specifically the risk of venous thrombosis linked to AMI in hospital, ambulatory and internal medicine settings. CURRENT KNOWLEDGE AND KEY POINTS: The incidence of venous thromboembolism is higher in hospital than in ambulatory setting, albeit the latter remains significant. Stroke and affections leading to intensive care management represent conditions at great risk. Several mechanisms leading to a prothrombotic state have been identified, explaining the increased risk observed during relapses of pathologies specifically treated in internal medicine such as lupus erythematosus, Wegener granulomatosis, inflammatory bowel diseases and Behcet's disease. FUTURE PROSPECTS AND PROJECTS: Next to the pathophysiological understanding of venous thrombosis, the assessment of the specific thrombogenic burden of an AMI is an additive tool to screen medical patients at high risk. This systematic review of the literature shows uncertainties towards some risk factors as bedrest or acute inflammatory response. Taking into account the methodological difficulties inherent to prospective and epidemiological studies, a meta-analysis focusing on these factors would be useful to refine prevention guidelines for venous thromboembolism in medical setting.
Subject(s)
Ambulatory Care/statistics & numerical data , Hospitals/statistics & numerical data , Internal Medicine/statistics & numerical data , Venous Thromboembolism/epidemiology , Venous Thromboembolism/etiology , Bed Rest , Behcet Syndrome/complications , Critical Care , France/epidemiology , Granulomatosis with Polyangiitis/complications , Humans , Incidence , Inflammatory Bowel Diseases/complications , Lupus Erythematosus, Systemic/complications , Mass Screening , Odds Ratio , Prospective Studies , Risk Assessment , Risk Factors , Stroke/complications , Venous Thromboembolism/physiopathologyABSTRACT
PURPOSE: Some acute medical illnesses are associated with an increased risk of venous thromboembolism. This risk is difficult to assess in medical patients on account of the multiplicity of their pathologies, leading to potential interactions between numerous risk factors. CURRENT KNOWLEDGE AND KEY POINTS: A rational analysis of the risk of venous thrombosis in acute medical setting may be derived from pathogenic models and be based on elementary risk factors for venous thromboembolism classified as transient, permanent, major and intermediate factors. The systematic study of pathophysiological mechanisms in process, in each medical patient could guide the physician attitude toward still non-evaluated situations. FUTURE PROSPECTS AND PROJECTS: A synthesis of the last epidemiological and pathophysiological data is developed in the first part of this literature review, whereas the risk linked to specific situations in ambulatory, hospital or internal medicine settings will be described in the second part.
Subject(s)
Acute Disease , Blood Circulation , Blood Coagulation Factors , Endothelium, Vascular/physiopathology , Venous Thromboembolism/epidemiology , Venous Thromboembolism/physiopathology , Evidence-Based Medicine , France/epidemiology , Humans , Incidence , Inflammation/physiopathology , Odds Ratio , Prospective Studies , Risk Assessment , Risk FactorsABSTRACT
BACKGROUND: Inflammatory involvement of extracranial large-sized arteries occurs in 10-20% of patients with giant cell (temporal) arteritis. Aortic involvement may reveal giant cell arteritis or occur as a late-onset complication, and represents one of the most serious manifestation of the disease with the risk of aortic dissection and/or aneurysm rupture. The thoracic aorta is more frequently involved but abdominal aortitis may also occur in giant cell arteritis. To date, few data are available about abdominal aorta changes at the initial stage of giant cell arteritis. PATIENTS AND METHODS: This prospective monocentric study was conducted between May 1998 and May 2002, and included 30 consecutive patients with biopsy-proven giant cell arteritis. Standard clinical and biological data were collected. Each patient underwent an abdominal aortic Doppler-sonography that looked for aneurysm, ectasia, thickening of the vascular wall, and hypoechoic halo around the aorta. RESULTS: Among the 30 patients of this study (25 women, 5 men, mean age 68.5 years), 4 (13%) had an abdominal aortic aneurysm, with a low diameter (23 to 27 mm), measuring 2 to 5.5 cm in length. A vascular wall thickening superior or equal to 3 mm was noted in 17 patients (68%). A 4 to 8 mm periaortic hypoechoic halo was found in 10 patients (33%). This halo was present in 3 out of the 4 patients with aneurysm. CONCLUSION: Aortic involvement is a potentially serious complication of giant cell arteritis. The question of a systematic screening of this complication remains open to discussion. Our study shows that Doppler sonography may detect morphological abnormalities on the abdominal aorta at the initial stage of giant cell arteritis. These abnormalities comprise mild aneurysms, thickening of the vascular wall and periaortic halo, which could correspond to inflammatory locations of the disease. Complementary studies are needed to assess their specificity and their seriousness.
Subject(s)
Aorta, Abdominal/diagnostic imaging , Giant Cell Arteritis/diagnostic imaging , Aged , Aged, 80 and over , C-Reactive Protein/metabolism , Diagnosis, Differential , Female , Humans , Male , Middle Aged , Prospective Studies , Ultrasonography, DopplerABSTRACT
Werner syndrome (i.e., adult progeria) is a rare autosomal recessive disorder caused by mutations of the WRN gene, which is characterized by the premature appearance of features associated with normal aging and cancer predisposition. Patients with Werner syndrome can present with musculoskeletal complaints, associated with suggestive radiographic features with a potential prognostic or therapeutic impact. This review illustrates the main radiographic features of Werner syndrome, focusing on the musculoskeletal system, such as soft-tissue calcification, muscular atrophy, osteoporosis, foot deformities, osteitis and osteomyelitis, and bone or soft-tissues malignancies. The identification of these features by radiologists can therefore be useful in the clinical screening of Werner syndrome.
Subject(s)
Musculoskeletal Diseases/diagnostic imaging , Musculoskeletal Diseases/etiology , Werner Syndrome/complications , Humans , RadiographyABSTRACT
The pathophysiology of primary Raynaud's phenomenon (Raynaud's disease) remains uncertain but the transmission of this primary microcirculatory dysregulation seems strongly influenced by genetic factors. For a long time, physicians have found that the hereditary factor plays an important role in the genesis of Raynaud's disease. Familial analysis and twin studies have confirmed the role of an hereditary factor. It seems heterogeneous but pedigree analysis indicates the possibility of an autosomal dominant transmission influenced by sex, in some families, allowing an approach called "reverse genetic" based on linkage analysis. Such an approach has focused on few loci but sequencing of candidate genes for genetic mutations remains negative. Given the supposed heterogeneity of the genetic transmission of Raynaud's disease, diversification of strategies in molecular genetics is suitable with reference to techniques applied to multifactorial heredity.
Subject(s)
Raynaud Disease/genetics , Female , Humans , Male , Microcirculation , PedigreeABSTRACT
PURPOSE: Clinical reasoning and treatment challenges within the scope of general practice led to the development of an internal medicine assistance line provided by Nantes University Hospital. The primary outcome of this study was to describe callers' profile, their requests and answers provided. METHODS: A prospective, cross-sectional, observational, descriptive study was undertaken. For each call were identified the calling physician, her/his specialty and work setting, the call's object and adequacy, the answer provided, the time needed to connect with the assistance line, the time devoted by the internal medicine physician to provide an answer to the request, and whether the assistance line prevented a visit to the emergency room. Each calling physician was then called back to obtain demographic and professional characteristics, and data relating to the call and to the assistance line. RESULTS: Sixty-three days were analyzed and 276 calls identified. The 237 identified calling physicians were mainly females (54%, n=93), with a mean age of 46 years, graduated from Nantes University (65%, n=86), practicing ambulatory general medicine (69%, n=164) in Loire-Atlantique department area (82%, n=176) for a mean duration of 15 years. Calls were mostly associated with diagnostic challenges (61%, n=166) concerning clinical issues (57%, n=155). A sole telephone advice was the main type of answer provided (56%, n=147) and a visit to the emergency room was prevented for 17% of calls. CONCLUSION: The assistance line activity is adequate with its missions and seems to facilitate patients' healthcare delivery advocating for the development of similar structures in other units. Improvements relating to the information, availability and physicians' training should be considered.
Subject(s)
General Practice , Hotlines , Internal Medicine , Telemedicine , Telephone , Adult , Aged , Clinical Decision-Making/methods , Cross-Sectional Studies , Disease , Female , France/epidemiology , General Practice/methods , General Practice/organization & administration , General Practice/standards , Hotlines/statistics & numerical data , Humans , Internal Medicine/methods , Internal Medicine/organization & administration , Internal Medicine/standards , Male , Middle Aged , Telemedicine/methods , Telemedicine/standardsABSTRACT
AIM: The physiopathology of Raynaud's phenomenon (RP) is not currently fully resolved. The cold seems to be not only an important factor triggering attacks, but also inducing RP. The aims of this study were to assess the prevalence of RP in Nantes urban district, and study the relationship between RP prevalence and cold climate. METHODS: Patients aged between 10 and 80 years old, consulting in five Nantes General Practices, from June 2011 and March 2012, were included. Patients presenting RP underwent a full clinical examination. Subjects not meeting Allen and Brown criteria benefited from at least a dosage of Anti-Nuclear Antibodies and a naifold Capillaroscopy. Climate data provided by French national weather agency allowed establishing an average of observed temperatures during the past five years and correlating them to the observed prevalence. RESULTS: Of 954 patients included, 78 had a RP, for an overall prevalence estimated at 8.2%. The prevalence among women (8.9%) was slightly higher than men (7.3%). Secondary form represented 5.1% of RP. In the RP group, 13 patients were active smokers, mean BMI was 22.3±3.2 kg/m², and only 4 patients were treated by vasoconstrictor therapy. According to French national weather agency, between 2007 and 2011, mean temperature of January in Nantes area was 5.8 °C. CONCLUSION: We confirmed that the lower winter temperatures a region experiences, the higher the prevalence of RP, thus raising the question of the physiopathological role of the cold in the induction or in the revelation of RP.
Subject(s)
Cold Climate/adverse effects , Fingers/blood supply , Raynaud Disease/diagnosis , Raynaud Disease/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Child , Female , France/epidemiology , Humans , Male , Microscopic Angioscopy , Middle Aged , Young AdultSubject(s)
Betacoronavirus/pathogenicity , Chilblains/epidemiology , Coronavirus Infections/epidemiology , Pneumonia, Viral/epidemiology , COVID-19 , Chilblains/diagnosis , Coronavirus Infections/diagnosis , Coronavirus Infections/therapy , Coronavirus Infections/virology , France/epidemiology , Humans , Pandemics , Pneumonia, Viral/diagnosis , Pneumonia, Viral/therapy , Pneumonia, Viral/virology , Prognosis , Risk Factors , SARS-CoV-2ABSTRACT
In the absence of established criteria enabling a Raynaud's phenomenon to be related to a thoracic outlet syndrome, the authors attempted to assess whether the presence of objective clinical features of thoracic outlet might modify the epidemiology or the clinical presentation of Raynaud's phenomenon (RP). From a population of 570 RP, 263 RP with primary feature were selected according to classical clinical, biological and capillaroscopy criteria. Within this group, apparently primary RP without any objective sign of thoracic outlet were compared with those presenting objective signs of thoracic outlet (ie positive "candlestick manoeuvre", abnormal dynamic Doppler examination). The two groups were compared according to epidemiologic (family history of RP, age of onset, sex), clinical (digital topography of attacks, asymmetry of RP, functional severity, course during summer, circumstances of occurrence) and investigational data (digital plethysmographic cold test). No significant differences were found between apparently primary RP with signs of thoracic outlet and those without signs of thoracic outlet as concerns the parameters usually distinguishing Raynaud's syndrome from Raynaud's disease (family history of vasospasm, sex, asymmetry of RP, thumb involvement, digital vascular reactivity to cold). On the other hand, significant differences are noted for the following parameters: age of onset (p = 0.005), course during summer (p = 0.031), circumstances of occurrence (p = 0.0007), digital topography of attacks (p = 0.004), functional severity (p = 0.044), plethysmographic basal reactivity (p = 0.02).(ABSTRACT TRUNCATED AT 250 WORDS)
Subject(s)
Raynaud Disease/epidemiology , Raynaud Disease/etiology , Thoracic Outlet Syndrome/complications , Thoracic Outlet Syndrome/epidemiology , Case-Control Studies , Female , Humans , Incidence , Male , Prospective Studies , Raynaud Disease/diagnosis , Thoracic Outlet Syndrome/diagnosisABSTRACT
The purpose of this study was to analyze the reactivity to cold in a series of 541 patients with Raynaud's phenomenon and to search for possible correlations with the functional severity and the etiologic diagnosis of the acrosyndrome. Digital reactivity to cold was quantified by a plethysmographic cold test performed under standardized conditions, using mercury-strain-gauge plethysmography. The functional severity of Raynaud's phenomenon was assessed by the weekly frequency of attacks during winter. Topographic evaluation of the acrosyndrome sought to determine the possible extension of attacks to the thumb. Factorial Analysis of Correspondences indicated a high correlation between the intensity of reactivity to cold and the frequency of thumb involvement (p = 0.0001) or the weekly frequency of attacks during winter (p < 10(-4)). There was also a close dependence between reactivity to cold and the etiologic diagnosis of the acrosyndrome (p < 10(-4)). Significant correspondences were found between mild or major types of reactivity and primary Raynaud's disease, between very severe reactivity to cold and scleroderma. This study contributes to a clinical validation of the plethysmographic cold test. During Raynaud's phenomenon, it is indicative of clinical severity and can in a certain way guide the etiologic diagnosis, particularly concerning the risk of scleroderma.
Subject(s)
Cold Temperature , Plethysmography , Raynaud Disease/diagnosis , Adolescent , Adult , Aged , Aged, 80 and over , Child , Factor Analysis, Statistical , Female , Fingers/blood supply , Humans , Male , Middle Aged , Raynaud Disease/etiology , Scleroderma, Systemic/complicationsABSTRACT
Many authors consider that late onset is a suspect criterion for differentiating primary Raynaud's phenomenon (Raynaud's disease, RD) from Raynaud's syndrome (RS). However, many cases of late-onset Raynaud's phenomenon in patients over forty years of age remain without etiologic diagnosis and therefore deserve the designation "late-onset RD." One hundred and ninety-four patients with RD (143 women, 51 men) were selected among 424 patients with Raynaud's phenomenon, according to Allen and Brown's criteria with negative serologic investigations and normal capillaroscopy. The purpose of the study was to consider the possible discriminant value of age of onset in distinguishing between RD and RS. The following epidemiologic features were studied: age of onset, sex, family history of Raynaud's phenomenon and migraine, and smoking and working habits. Microcirculation was assessed by capillaroscopy and strain-gauge plethysmography. Maximal digital flow at 45 degrees C and reactivity to cold were determined for each patient. Results were related to age of onset. The existence of true cases of late-onset RD in patients over forty years of age was confirmed (prevalence 27%), showing a correlation with a family history of Raynaud's phenomenon inferior to that found in early-onset cases (p < 0.0001). Microcirculation studies generally indicated a strong correlation between reactivity to cold, familial RD, and early onset, whereas no correlation was found with migraine or smoking. Nor was there any clinical or plethysmographic evidence for arteritis as a possible pathogenetic factor in late-onset RD. These results indicate that late-onset RD is a valid designation and that its pathogenesis seems less dependent on genetic sensitivity to cold than that of early-onset cases. In the absence of underlying arteritis, neurovascular dysfunction or a hemorheologic mechanism may be suggested as plausible causes of late-onset RD.
Subject(s)
Raynaud Disease/epidemiology , Adult , Age Distribution , Age of Onset , Cold Temperature/adverse effects , Diagnosis, Differential , Female , Fingers/blood supply , Humans , Male , Microcirculation/physiopathology , Middle Aged , Plethysmography , Prevalence , Prospective Studies , Raynaud Disease/etiology , Raynaud Disease/genetics , Risk Factors , Sex FactorsABSTRACT
The authors' aim was to assess, using a routinely usable methodology, the distal vasomotor reflexes induced by postural changes of the lower limbs in 36 healthy volunteers (18 women and 18 men, mean age: 22.3 years, range 19-26). These distal vasomotor changes were recorded on the great toe using mercury-strain-gauge plethysmography. In the first step, the authors have measured the veno-arterial reflex obtained by elective lowering of the leg, the subject lying strictly horizontally under standardized conditions. Secondly was assessed the distal vasomotor reflex due to orthostatism (seated position, legs hanging). Correlations between these postural reflexes and height, weight, sex, length of the lower limbs, femoral venous diameters assessed by echography were studied. The veno-arterial reflex obtained by elective verticalization of the leg (0.68 +/- 0.21) corresponds to a 32% mean decrease in the amplitude of the plethysmographic recording as compared to the initial amplitude in horizontal position. It is all the more marked as the subject is male and the leg is long, thus inducing a equivalent change of hydrostatic venous pressure during verticalization of this part of the limb. On the other hand, this adaptative reflex appears all the more intense as the basal vascular tone in horizontal position is low. Passing to orthostatism induces a distal vasoconstriction corresponding to a 51% mean decrease in the amplitude of the plethysmographic signal as compared to the initial basal amplitude. This general reflex, playing a part in the keeping of hemodynamic conditions when passing to orthostatism, appeared significantly correlated to the diameter of the common femoral vein when the subject is lying horizontally.