Favourable outcome of planned pregnancies in systemic sclerosis patients during stable disease.

OBJECTIVE: Studies evaluating pregnancy outcomes in systemic sclerosis (SSc) are limited. SSc is associated with maternal complications and adverse neonatal outcomes. This study investigated the impact of disease stage (stable vs active) on the maternal and neonatal outcomes of pregnancies of patients followed at an Israeli medical centre. METHOD: The charts of 354 SSc female patients followed during 2003-2020 were reviewed. Data on clinical and laboratory features, number of pregnancies close to SSc diagnosis, and maternal and neonatal outcomes were analysed. Patients were divided into a stable disease and an active/early disease group. RESULTS: The active/early disease group included 26 patients [19 diffuse SSc (dSSc)], with 38 pregnancies. Median disease duration was 1 year, except for four patients who were first diagnosed during pregnancy. SSc was exacerbated in all patients during pregnancy or shortly after delivery [skin, lung, and heart involvement in dSSc; severe vasculopathy in limited SSc patients]. Six patients had hypertensive disorders of pregnancy; four pregnancies ended with foetal death. Thirty-three children were born, 60% with intrauterine growth retardation (IUGR)/low birthweight (LBW). The stable disease group included 19 patients, including seven with previously active disease, now stabilized (five dSSc), and 32 pregnancies. All pregnancies were planned and monitored closely. Disease remained stable in all patients. Four patients had hypertensive disorders of pregnancy; 12/29 newborns had LBW (41%). CONCLUSION: Active maternal disease during pregnancy poses an increased risk of SSc aggravation. The maternal and neonatal outcomes in planned pregnancy during stable disease are favourable. IUGR/LBW is common among neonates, even in stable disease.

Differences associated with age at onset in early systemic sclerosis patients: a report from the EULAR Scleroderma Trials and Research Group (EUSTAR) database.

OBJECTIVE: The aim of this study was to analyse differences in clinical presentation in patients with early (< 3 years' duration) systemic sclerosis (SSc), comparing three age groups according to disease subsets. METHOD: Cross-sectional analysis of the prospective EULAR Scleroderma Trials and Research database (EUSTAR) was performed. Patients fulfilling preliminary American College of Rheumatology 1980 classification criteria for SSc, with < 3 years from the first non-Raynaud's SSc symptom at first entry, were selected. Patients with < 3 years from the first SSc symptom, including Raynaud's phenomenon, were also analysed. SSc-related variables, including antibodies, SSc subsets, and organ involvement, were examined. Age was categorized into ≤ 30, 31-59, and ≥ 60 years. We performed descriptive and bivariate analyses. RESULTS: The study included 1027 patients: 90% Caucasian, 80% women, and 40% with diffuse disease. In early stages of SSc, younger patients had significantly more anti-Scl-70 antibodies and diffuse disease. With increasing age, we observed more elevation of estimated pulmonary systolic pressure on echocardiography (5%, 13%, and 30%, respectively, in the three age groups), cardiac conduction blocks (6%, 6%, and 15%), and left ventricular diastolic dysfunction (4%, 12%, and 27%). The results were similar for 650 patients with < 3 years from first SSc symptom, including Raynaud's. CONCLUSION: In early stages of SSc, older patients showed data indicating more severe disease with greater cardiac involvement. The diffuse subset was more frequent in the younger subgroup. The identification of such differences may help in selecting appropriate management for individual patients in clinical practice.

Felty's syndrome without rheumatoid arthritis?

Felty's syndrome (FS) is characterized by neutropenia and splenomegaly in patients with seropositive (RF+, anti-CCP+) rheumatoid arthritis (RA). As a result of neutropenia, affected persons are increasingly susceptible to infections. In the majority of patients, FS appears in the course of long-standing and well-established RA. Manifestations of FS without clinical but only with laboratory features of RA are extremely rare. We present a case of severe neutropenia and mild splenomegaly in a patient with high titers of RF and anti-CCP. For 4 years, patient's neutropenia remained asymptomatic. The neutropenia reduction to agranulocytosis was followed by successful methotrexate-corticosteroid therapy. Efficacy of the standard anti-RA therapy confirmed autoimmune mechanism of the Felty's neutropenia. The most important lesion from our case is to recognize this condition in the range of autoimmune rheumatic diseases without delay. We reviewed literature with non-articular FS.

Pneumatosis cystoides intestinalis in scleroderma-related conditions.

AIMS: Pneumatosis cystoides intestinalis (PCI) is a rare life-threatening gastrointestinal complication in the course of connective tissue disease (CTD). PCI is characterised by the appearance of intramural clusters of gas in the small and large bowel wall on X-ray or computed tomography and often is accompanied by free air in the peritoneal cavity. METHODS: We present three cases of PCI in patients with scleroderma-related conditions. A review of the English language literature published on MEDLINE from 1973 to 2008 was conducted using the terms: 'systemic sclerosis', 'connective tissue disease' and 'pneumatosis cystoides intestinalis'. This review focused on clinical features, diagnostic and treatment strategies of PCI in the context of CTD. RESULTS: Symptoms of PCI are non-specific: abdominal pain, vomiting, constipation, bloating and weight loss. Coexistence of PCI with other manifestations of CTD, such as intestinal pseudo-obstruction and/or bacterial overgrowth, complicates the clinical diagnosis. Treatment approach to PCI is mostly conservative: intestinal 'rest', parenteral nutrition, antibiotics, fluids and electrolyte supplementation, and inhaled oxygen. Surgical intervention should be performed only in cases of bowel perforation, ischaemia or necrosis. Patients with PCI have high mortality rates due to PCI itself but also to the severity and variety of basic CTD complications. CONCLUSION: Recognition of PCI, particularly in the context of underlying CTD, is necessary for proper therapeutic application. In patients with underlying CTD and symptoms of abdominal emergency, recruitment of multidisciplinary teams, including rheumatologist, gastroenterologist, imaging specialist and surgeons familiar with intestinal complications of CTD-related conditions, is warranted.

Clinical prediction of 5-year survival in systemic sclerosis: validation of a simple prognostic model in EUSTAR centres.

OBJECTIVE: Systemic sclerosis (SSc) is associated with a significant reduction in life expectancy. A simple prognostic model to predict 5-year survival in SSc was developed in 1999 in 280 patients, but it has not been validated in other patients. The predictions of a prognostic model are usually less accurate in other patients, especially from other centres or countries. A study was undertaken to validate the prognostic model to predict 5-year survival in SSc in other centres throughout Europe. METHODS: A European multicentre cohort of patients with SSc diagnosed before 2002 was established. Patients with SSc according to the preliminary American College of Rheumatology classification criteria were eligible for the study when they were followed for at least 5 years or shorter if they died. The primary outcome was 5-year survival after diagnosis of SSc. The predefined prognostic model uses the following baseline variables: age, gender, presence of urine protein, erythrocyte sedimentation rate (ESR) and carbon monoxide diffusing capacity (DLCO). RESULTS: Data were available for 1049 patients, 119 (11%) of whom died within 5 years after diagnosis. Of the patients, 85% were female, the mean (SD) age at diagnosis was 50 (14) years and 30% were classified as having diffuse cutaneous SSc. The prognostic model with age (OR 1.03), male gender (OR 1.93), urine protein (OR 2.29), elevated ESR (1.89) and low DLCO (OR 1.94) had an area under the receiver operating characteristic curve of 0.78. Death occurred in 12 (2.2%) of 509 patients with no risk factors, 45 (13%) of 349 patients with one risk factor, 55 (33%) of 168 patients with two risk factors and 7 (30%) of 23 patients with three risk factors. CONCLUSION: A simple prognostic model using three disease factors to predict 5-year survival at diagnosis in SSc showed reasonable performance upon validation in a European multicentre study.

Vitamin D level: is it related to disease activity in inflammatory joint disease?

The objectives of this study are to assess the vitamin D status in patients (pts) with inflammatory joint diseases (IJD), and its correlation with disease activity. 121 consecutive pts (85 rheumatoid arthritis (RA), 22 psoriatic arthritis (PSA), 14 ankylosing spondylitis (AS)) underwent clinical and laboratory evaluation which included kidney and liver function tests, serum calcium and phosphor levels, 25(OH)D and parathyroid hormone (PTH). Disease activity was assessed by DAS 28 in RA and PSA pts and by BASDAI in AS pts, sedimentation rate (ESR) and CRP. According to activity indexes, pts were divided into subgroups with low (DAS28 < 3.2 and BASDAI < 4), and moderate-to-high disease activity (DAS28 > 3.2 and BASDAI > 4). Associations between serum levels of 25(OH)D and age, gender, ethnicity, type and disease duration, treatment, (anti-tumor necrosis factorα (TNFα) agents or DMARDs), seasonal variations, and disease activity were assessed. Vitamin D deficiency was found in 51 pts (42.1%). The incidence was higher among Arab pts (76.7%) compared to Jews (23%). The difference of 25(OH)D levels between Arabs (mean 9.4 ± 4.2 ng/ml) and Jews (mean 17.8 ± 8.4 ng/ml) was statistically significant (p < 0.0001). We did not find correlation between vitamin D levels and the other evaluated factors. A surprisingly high incidence of vitamin D deficiency was found in IJD patients in a sunny Mediterranean country. This finding justifies the inclusion of vitamin D in the routine lab work-up of pts with IJD. The only statistical significant correlation was found between vitamin D level and ethnic origin. Further studies are needed to look for genetic polymorphism of vitamin D receptors.

Seronegative polyarthritis as severe systemic disease.

BACKGROUND: Severe extra-articular disease is associated with high levels of rheumatoid factor (RF ) in patients with seropositive rheumatoid arthritis (RA ) and a poor prognosis. It is said that patients with seronegative rheumatoid arthritis have a more benign course and less destructive disease. We observed several patients with seronegative non-rheumatoid polyarthritis, with aggressive extra-articular systemic disease. OBJECTIVES: Review of seronegative systemic polyarthritis with clinical presentation of typical cases. METHODS: Medline search for systemic manifestations of seronegative polyarthritis. CLINICAL PRESENTATIONS: 1. A 56-year-old woman was admitted to the cardiac intensive care unit with stabbing presternal chest pain aggravated by breathing and progressive dyspnoea, which gradually developed over a period of two weeks with one episode of fever at 38.0 degrees C. She had suffered chronic pain in her buttocks for three years with polyarthralgia and evanescent palmar-plantar rash. Imaging showed bilateral sacroiliitis (HLA B27 negative) and a large pericardial effusion. Extra-articular manifestations of SAPHO syndrome were proposed and she was successfully treated with combined therapy: pulse methylprednisolone, azathioprine, colchicine and prednisone. 2. A 47-year-old woman with psoriatic arthropathy developed high fever with leucocytosis and thrombocytosis and lung infiltrates during exacerbation of her joint disease . She was treated with pulse methylprednisolone followed by corticosteroid tapering, anti-TNF (infliximab) and methotrexate with complete resolution. 3. A 19-year-old man with inflammatory bowel disease developed acute pericarditis with response to 6-mercaptopurine, salazopyrine and prednisone. RESULTS: We discuss a range of seronegative arthritis diseases with possible systemic manifestations including the main procedures for early diagnosis. Infection, malignancy, hypersensitivity, granulomatous disease and other collagen diseases such as systemic lupus erythematosus should be excluded, but investigations for an underlying disease should not delay early corticosteroid and immunosuppressive therapy. CONCLUSION: A high level of suspicion of extra-articular disease should always be maintained when treating active seronegative polyarthritis.

Vaccination against influenza in patients with rheumatoid arthritis: the effect of rituximab on the humoral response.

OBJECTIVE: To assess the effect of rituximab on the efficacy and safety of influenza virus vaccine in patients with rheumatoid arthritis (RA). METHODS: The study group comprised patients with RA treated with conventional disease-modifying drugs with or without rituximab. Split-virion inactivated vaccine containing 15 microg haemagglutinin/dose of B/Shanghai/361/02 (SHAN), A/New Caledonian/20/99 (NC) (H1N1) and A/California/7/04 (CAL) (H3N2) was used. Disease activity was assessed by the number of tender and swollen joints, duration of morning stiffness and evaluation of pain on the day of vaccination and 4 weeks later. CD19-positive cell levels were assessed in rituximab-treated patients. Haemagglutination inhibition (HI) antibodies were tested and response was defined as a greater than fourfold rise 4 weeks after vaccination or seroconversion in patients with a non-protective baseline level of antibodies (<1/40). Geometric mean titres (GMT) were calculated in all subjects. RESULTS: The participants were divided into three groups: RA (n = 29, aged 64 (12) years), rituximab-treated RA (n = 14, aged 53 (15) years) and healthy controls (n = 21, aged 58 (15) years). All baseline protective levels of HI antibodies and GMT were similar. Four weeks after vaccination, there was a significant increase in GMT for NC and CAL antigens in all subjects, but not for the SHAN antigen in the rituximab group. In rituximab-treated patients, the percentage of responders was low for all three antigens tested, achieving statistical significance for the CAL antigen. Measures of disease activity remained unchanged. CONCLUSION: Influenza virus vaccine generated a humoral response in all study patients with RA and controls. Although the response was significantly lower among rituximab-treated patients, treatment with rituximab does not preclude administration of vaccination against influenza.

Intravenous immunoglobulins in polyarteritis nodosa restricted to the limbs: case reports and review of the literature.

Polyarteritis nodosa (PAN) of the calf muscles is a rare form of vasculitis. We present two cases of PAN limited to the calf and a review of the literature, based on a MEDLINE (PubMed) search of the English literature from 1980 to 2005, using the key words "vasculitis restricted to limbs", "polyarteritis nodosa", and "intravenous immunoglobulin". PAN limited to the calf muscles is a condition presenting with severe shin pain and walking difficulties. In contrast to classic PAN, there is no skin, joint, visceral or nerve system involvement in this form of the disease. The main clinical signs are tenderness and swelling of the calf. Inflammatory markers, such as erythrocyte sedimentation rate and C-reactive protein, are usually elevated, and a perinuclear pattern of anti-neutrophil cytoplasm antibodies can be found. Electromyography of the calf is not contributory. Magnetic resonance imaging may be useful in recognizing the limb-restricted vasculopathy and selecting the muscle biopsy site, which is obligatory for diagnosis. Corticosteroids (CS) are the main treatment regimen, but CS-resistant cases have been reported. The patients presented here failed to respond to CS but were successfully treated with intravenous immunoglobulin therapy (IVIG). In the absence of vital organ involvement, the addition of cytotoxic drugs is controversial. IVIG seems to be an efficient alternative therapy in PAN limited to the calf muscles especially for patients with limitations to conventional cytotoxic treatment.

Anterior sonography of glenohumeral joint in patients with inflammatory joint disease.

Ultrasonography (US) was shown as an effective imaging modality in evaluating the shoulder. The shoulder joint is probably the most accessible joint for sonography in adults. However, inflammatory changes of the shoulder have received too little attention in US studies. Anterior access for US assessment of glenohumeral joint (GHJ) has not been investigated. Another problem of patients with acute synovitis of glenohumeral joint is the difficulty to perform a 90 degrees abduction for the axillary US because of severe pain and active and passive limitation. We offer the anterior access for assessment of glenohumeral joint synovitis (GHS). Sonographic evaluation (Sonosite-Titan) was carried out in 25 patients with acute GHS and 15 healthy controls. The diagnosis of GHS was made after the patients underwent physical examination and the laboratory evidence was obtained. We used the anterior position of transducer applied laterally to coracoid processus along the anterior joint cavity. The problem of anterior joint cavity investigation in neutral position is a poor presentation of the joint and the application of the biceps tendon. The problem is simply resolved after supination of the hand and external rotation of the shoulder. We measured and compared upper, middle, and lower width of the anterior GHJ cavity. Echogenicity of joint cavity was assessed by comparison with adjacent tissues. Homogeneity and regularity of GHJ cavity was designated in both groups as well. We measured labrum-infraspinatus distance on posterior view for assessment of GHJ synovitis. All cases of GHJ synovitis were confirmed by a US Doppler study. US investigation of healthy controls enabled to find normal values of the width of the anterior GHJ cavity that was less than 7.4 mm. The synovitis group showed GHJ cavity expansion: 8.3+/-2.4 (p=0.001) and 10.5+/-3.1 (p<0.001) for the middle and the lower anterior part of the GHJ respectively. The upper part width was not different in synovitis and control groups. Anterior joint cavity extension to 7.4 mm and upper in its lower part was high sensitive (96%) and specific (86%) US sign of synovitis with the test power above 0.9. The posterior labrum-infraspinatus extension had high specificity for synovitis (100%), but only seven of 25 patients (28%) had increased (>2 mm) the value of the labrum-infraspinatus dimension, which was previously proposed as the US sign of synovitis. Echogenicity of the anterior joint cavity in healthy controls was moderately high (far more echogenic than deltoid muscle). Echogenicity of synovitis declined, and mild effusions were found to be common. Those were not to be seen on US of GHJ in neutral position and were revealed only in supination and external rotation of the shoulder. Intra-articular tissue of healthy controls was relatively echo-homogenic compared with nonhomogenic one of the synovitis group. Bone irregularity was found in patients with long-standing GHJ synovitis reflecting erosive process. A certain position of the shoulder and good knowledge of the normal anterior joint cavity parameters enabled us to diagnose synovitis by anterior shoulder sonography, with the patients experiencing minimal pain during movements.

Sustained benefit in rheumatoid arthritis following one course of rituximab: improvements in physical function over 2 years.

OBJECTIVES: To evaluate the long-term impact on physical function of a single course of rituximab in rheumatoid factor, seropositive patients with active rheumatoid arthritis (RA) despite ongoing methotrexate treatment. METHODS: A randomized, controlled trial comparing rituximab alone [1,000 mg intravenously (iv) on days 1 and 15, n= 40], or in combination with cyclophosphamide (750 mg iv on days 3 and 7, n= 41) or oral methotrexate (> or =10 mg/week, n= 40) with placebo + methotrexate (> or =10 mg/week, n= 40), resulted in significant reductions in disease activity at weeks 24 and 48. Sustained improvements in physical function and standard effect sizes (SES) for changes in components of ACR and EULAR criteria were evaluated over 2 yrs. RESULTS: More patients receiving rituximab + methotrexate completed a 2-yr follow-up without further treatment than those receiving placebo + methotrexate (45% vs 15%, respectively), rituximab alone (10%) or rituximab + cyclophosphamide (22%). This reflected a higher percentage of patients receiving rituximab + methotrexate reporting improvements in Health Assessment Questionnaire Disability Index > or = minimum clinically important difference at 1 and 2 yrs (68% and 30%, respectively) compared with placebo + methotrexate (28% and 15%), rituximab monotherapy (43% and 10%) or rituximab + cyclophosphamide (39% and 12%). SES were high in all rituximab groups and revealed differing patterns of response over time. CONCLUSION: A single course of rituximab with continuing methotrexate in patients with active RA provided clinically meaningful improvements in physical function over 2 yrs, with lower discontinuation rates and larger SES for improvements in ACR and EULAR criteria components.

Essential mixed cryoglobulinemia type II.

We report a rare case of essential mixed cryoglobulinemia type II with membrano-proliferative glomerulonephritis (MPGN) type I in which HCV was not found. Long-term history of palindromic rheumatism, skin leukocytoclastic vasculitis attacks and micro-normocytic anemia preceded the appearance of cryoglobulinemia. Cryoprecipitate consisted of monoclonal IgMk-RF and polyclonal IgG (essential mixed type II). The newly appreciated cryoglobulinemia was associated with Coombs positive hemolytic anemia. The MPGN in this case had a benign course and responded to complex simple therapies including prevention of exposure to cold, low antigen content diet, treatment of provoking factors such as UTI, and maximal dose of ACE inhibitor. Responsiveness of skin vasculitis to colchicine therapy was restored after a two-month colchicine withdrawal period and therefore corticosteroid and immunosuppressive therapy was postponed.

Serum-synovial gradient data of normouricemic patients with history of gout and acute knee effusion.

The etiology of arthritis episodes in normouricemic patients with gout is still unclear. We propose that the fluctuation in synovial urate level, as well as pH, ion strength, albumin, and globulin values relative to serum levels, could be involved in crystal formation. To assess serum-synovial gradient (SSG), the sera and synovial fluid (SF) of six normouricemic patients (men, age 48-79) with a history of gout (American College of Rheumatology criteria) and acute knee effusion were screened for uric acid, pH, osmolality (Osm), P/Ca, albumin, globulin, and SSG. Monosodium urate monohydrate (MSUM) crystals were determined by polarized light (PL). Infectious arthritis was ruled out via Gram staining and synovial fluid culture. Negative X-ray and PL microscopy results excluded chondrocalcinosis. Five patients (1-5) had inflammatory knee effusion (WBC >2,000/mm(3)), and one (patient 6) had noninflammatory knee effusion (600 WBC/mm(3)). MSUM crystals were found in the WBC of patient 1 only. He had tophaceous gout with normal serum uric acid levels and showed significant negative Osm and P and positive Ca SSG. Two crystal negative patients had severe negative pH SSG with alkaline synovial fluid, significant P/Ca SSG, and high positive globulin SSG, while one of them had supersaturated SF uric acid content. The other patients displayed an increased Osm and P/Ca SSG. All SSG values were five to ten times higher than the coefficient of variance for used methods. Noninflammatory SF of patient 6 does not appear to be related to active gout. The data on SSG for MSUM, pH, Osm, Alb/Glob, and P/Ca in normouricemic patients with gout history and acute knee effusion was not homogeneous. We propose that acid-base and ionic-protein gradient may lead to instability of subsaturated urate solution, thereby predisposing to MSUM deposits within synovial membrane and inducing inflammation.

Recalcitrant leg ulcer due to mixed connective tissue disease.

We present a 28-year-old woman with mixed connective tissue disease (MCTD) complicated by a recalcitrant longstanding leg ulcer, which responded to complex therapy with local polydine, systemic ciprofloxacin, iloprost, enoxaparin and aspirin. Cyclophosphamide pulse therapy and corticosteroids controlled the systemic inflammation but failed to heal the leg ulcer. We considered a rationale of complex therapy for the leg ulcer on a basis of pathogenesis and complications of MCTD.

Seasonal distribution of relapse onset in rheumatoid arthritis and spondyloarthropathy: the possible effect of the solar factor.

BACKGROUND AND OBJECTIVE: The seasonal effect on the relapse of rheumatoid arthritis and spondyloarthropathies is still unclear. To assess the seasonal distribution of relapse onset in rheumatoid arthritis (RA) and spondyloarthropathy (SpA) and its association with solar factors. METHODS: The monthly distribution of relapse onsets during the years 1998-2000 was retrospectively chart reviewed in 364 patients. In 1998 a total of 131 patients were studied; 60 with seropositive (sp) RA, 30 with seronegative (sn) RA and 41 with SpA; 113 patients in 1999: 44 with spRA, 38 with snRA and 31 with SpA; 120 patients in 2000: 56 with spRA, 38 with snRA and 26 with SpA. All of them were treated in the Department of Rheumatology, which serves the population of northwestern Israel. Solar activity was analyzed according to the "Solar Terrestrial Activity Report Charts 1998-2000". The Central Israel Bureau of Statistics provided the sun global radiation data. Data was assessed during the summer (April-September) and winter (January-March, October-December). The correlation between the monthly distribution of disease relapses and solar factors was measured (SPSS-10 for WIN). RESULTS: Relapses in spRA patients occurred mostly during the summer months with peak activity during the month of July 2000. Single monthly peaks of spRA relapse onset were noted in January 1998-1999 and April 1998 and for snRA in January 1998 and June 2000, but there were no seasonal differences for spRA, snRA and SpA in 1998-1999 and for snRA and SpA in 2000. Relapses in spRA patients were associated with a summer bias of increased solar activity and global solar radiation in 2000 compared with lower peak solar activity in 1998-1999. Furthermore, in 2000 we found a significant correlation of the spRA monthly relapse count to solar activity (p = 0.005) and global sun radiation (p = 0.048) unlike snRA and SpA. No above-mentioned association and correlation was noted in 1998-1999. We revealed mild negative correlation (p = 0.046) of SpA relapse count only to peak solar flux (PSF) by analysis of data for 1998-2000 as one united group. CONCLUSIONS: Relapses were more frequent during the summer of 2000 (May-June-July) in spRA but not in snRA and SpA. The reasons are still unclear. No seasonal differences were observed in 1998-1999. Enhanced solar activity in summer-2000 compared with 1998-1999 may be inferred to be the proposed cause but coincidence may occur as well. Outbreak in RA and SpA was not registered despite increased peak solar activity in 2000. We observed mild evidence of reciprocal relation between SpA relapsing and solar activity during 1998-2000. Solar and any other possible contributory factors remain still to be elucidated.

Non-invasive measurement of biomechanical skin properties in systemic sclerosis.

OBJECTIVE: To evaluate biomechanical properties of skin in patients with systemic sclerosis (SSc) using the BTC-2000 suction device. METHODS: Twenty five patients with limited cutaneous SSc (lcSSc), 20 patients with diffuse disease (dcSSc), and 25 age matched healthy controls were evaluated. Viscoelastic deformation (VED, mm), elastic deformation (ED, mm), ultimate deformation (UD, mm), and pressure-deformation ratio (PDR, mm Hg/mm) were measured on the dorsal surface of the forearm, shoulder, and above the trapezius muscle on the back. RESULTS: Indices of skin extensibility (VED, ED, UD) were reduced and resistance to stress (PDR) increased in patients with dcSSc compared with healthy controls, or patients with lcSSc, at all three sites (p<0.001). At all sites, and overall, UD, ED, and VED were lower and PDR was higher at skin score above grade 2, compared with clinically normal skin. For both lcSSc and dcSSc biomechanical differences from controls were found even at sites of clinically normal skin. CONCLUSION: BTC-2000 is a sensitive tool for clinical evaluation of skin involvement in SSc and may be a valuable adjunct to skin sclerosis score in disease monitoring.

Cocaine-Induced raynaud's phenomenon and ischaemic finger necrosis.

A 37-year-old man with ischaemic finger necrosis and recent-onset Raynaud's phenomenon associated with cocaine abuse is reported. Initial therapy with systemic vasodilators, low-molecular-weight heparin and aspirin failed. Resolution of the ischaemia and ulcer healing was rapidly achieved with intravenous infusions of the prostacyclin analogue iloprost. The mechanism of vascular ischaemic injury and the development of secondary Raynaud's phenomenon due to cocaine use is discussed.