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The objective findings of phalangeal T2-weighted hyperintense and T1-weighted hypointense bone marrow signal on MRI without features of seronegative arthropathy or osteomyelitis may assist clinicians in making a diagnosis in the appropriate clinical context.

Case report - Introduction Waldenström’s macroglobulinemia (WM) is a rare haematological malignancy accounting for 2% of blood cancers. It is characterised by an immunoglobulin M (IgM)-associated lymphoplasmacytic lymphoma and usually follows an indolent disease course. Most patients present with nonspecific constitutional symptoms, commonly fatigue secondary to anaemia. Cryoglobulins are found in approximately 15% of WM patients but are symptomatic in less than 5% of cases. Symptoms range from Raynaud’s phenomenon to skin ulcers. We present a 56-year-old presenting with episodic blue discoloration of the ears and face, associated with cold exposure. Investigation revealed a diagnosis of WM with symptomatic cryoglobuloinaemia. Case report - Case description A 56-year-old female was referred to the rheumatology clinic with symptoms of Raynaud’s phenomenon. Further questioning revealed that she had been having Raynaud’s symptoms for many years, however over the last 18 months she experienced blue discolouration of her face, neck and ears when exposed to the cold. The lesions were itchy but non-tender and settled promptly on re-warming. There was no skin blistering or ulceration. She had also noted a reduced exercise tolerance. She was previously a keen runner but found she was short of breath after 2 miles, particularly on an incline or flight of stairs. There was no history of weight loss, loss of appetite or night sweats. Examination revealed skin changes consistent with resolving chilblains on the feet, with some nail dystrophy. There were a couple of splinter haemorrhages on one of the fingernails and no features of active Raynaud’s. There was no lymphadenopathy or hepatosplenomegaly and both cardiovascular and respiratory examination were normal. Blood tests showed a normal full blood count and renal function. Further investigations went on to reveal a cryoglobulinaemia with IgM kappa paraprotein. She was then referred to the haematology team who arranged for a bone marrow biopsy. This showed a 10—15% infiltration of low-grade B-cell lymphoma. A CT chest, abdomen, pelvis reported no lymphadenopathy, a normal spleen and a thyroid goitre. Treatment was initiated in the form of a triple therapy, dexamethasone, rituximab and cyclophosphamide. She underwent one cycle with good response and her original symptoms have now resolved. She is awaiting her second cycle of treatment. Case report - Discussion Lymphoplasmacytic lymphoma (LPL) is a mature B-cell neoplasm composed of small B lymphocytes showing plasmacytoid or plasma-cell differentiation. WM is a form of LPL in which there is evidence of bone marrow involvement and a monoclonal IgM paraprotein in the serum. WM accounts for over 95% of cases of LPL. Patients with WM can have symptoms caused by tumour infiltration (fever, night sweats, weight loss, fatigue) and/or by monoclonal IgM (hyperviscosity, cyoglobinaemia, cold agglutinin). Cryoglobulins are immunoglobulins or immune complexes (which contain rheumatoid factor) that precipitate below 37 °C and re-dissolve on warming. Typically, cryoglobulin measurements are done on serum samples using tubes without gel separators. Temperature control of the specimen during collection, transportation and centrifugation is vital and if not done correctly, can affect diagnosis. The term cyroglobulinaemia refers to the presence of one or more of these immunoglobulins in the serum. Cryoglobulinaemia in association with WM usually involve type I cryoglobulins or mixed immunoglobulin complexes, where monoclonal IgM behaves as an antibody against polyclonal IgG. Patients with symptomatic cryoglobulinaemia can present in a variety of ways including rapidly progressive glomerulonephritis, polyarthralgia, cutaneous ulcers and Raynaud’s phenomenon. In WM patients with symptomatic cryoglobulinaemia, exposure to cold leads to intravascular gelling of cryoglobulins with subsequent intravascular agglutination of red cells. This impairs the circulation to the cold exposed organs and vasoconstriction of the vessels can occur. The stagnant blood causes ischaemia and with pallor and consequently cyanosis develops. In our patient this pathway led to Raynaud’s and facial cyanosis symptoms. Case report - Key learning points Although the presence of cryoglobulins is frequent in WM, fewer than 5% are symptomatic. This case illustrates the importance of thorough history and investigation in patients that are referred to the Rheumatology outpatient clinic with presumed primary Raynaud’s phenomenon. Our patient had probable primary Raynaud’s phenomenon for many years, however she then had a change in symptoms, i.e., facial cyanosis, and this is what prompted further investigation for potential malignancy. The symptoms can be present for many months before the insidious nature of the underlying WM manifests and thus a high index of suspicion is necessary. Another important learning point is, if a patient presents with Raynaud’s symptoms and they do not meet the typical demographic for primary Raynaud’s disease (young onset, relatively mild disease with female preponderance) it is crucial to investigate for secondary causes. Skin manifestations related to symptomatic cryoglobulinaemia are common, with the most frequent being purpura and ulcers. Our patient presented with intermittent cyanosis of the face that could have progressed to necrotic ulceration if a diagnostic delay had occurred. Treatment directed at the underlying cause of the symptomatic cryoglobulinaemia results in improvement or stabilisation of symptoms in the majority of patients, with disappearance of cryoglobulins in over 50% of cases. Finally, clinicians often rely on constitutional symptoms of lethargy, weight loss and night sweats being present as triggers to investigate for a malignancy. Our patient displayed none of these red flag symptoms and even had a normal haemogloblobin level. This emphasises that awareness of this relatively rare disease entity is essential when faced with similar clinical scenarios as our patient and that cryoglobulinaemia should be on our differential list.

Primary Raynaud's phenomenon (PRP) manifests as episodes of transient spasms of peripheral blood vessels. To elucidate the clinical clues and laboratory characteristics will facilitate the identification of PRP.A retrospective data collection of clinical and laboratory characteristics of 58 children with PRP was performed between January 2007 and December 2016.A positive ANA test at lower titers1:100 was detected in 24.1% of the patients. There was a significant relationship between presence of ANA positivity and migraine in female patients with PRP (p=0.01; p=0.020 respectively). The most common accompanying disorder was migraine which was detected in 37.9% of all patients with PRP. Hemoglobin and serum ferritin levels were significantly lower in PRP patients with migraine (p=0.045; p0.05, respectively). Additionally, the mean platelet volume (MPV) measurements were significantly higher in patients with migraine compared to those without migraine (p=0.045; p0.05 respectively).There is limited data concerning childhood PRP. For the first time we showed a high frequency of migraine in childhood PRP. Anemia and high MPV could be the underlying triggering factors of these two episodic diseases.

Key Clinical Message We present a case of severe Raynaud phenomenon (RP) in an infant. The current strategy of RP treatment is incomplete; excluding secondary Raynaud phenomenon is vital as well. This case aims to help those with similar symptoms in the future by gathering data on cases.

Background Raynaud’s phenomenon (RP) is frequently associated with the presence of scleroderma or other connective tissue diseases (CTD). Identify the presence of secondary RP is important to perform an adequate therapeutic management and to achieve the early control of these patients. Nailfold capillaroscopy is safe, economic, easy to perform and has proven to be useful in identifying patients with secondary RP. Objectives To assess a long-term follow-up primary RP patient’s cohort. Methods Retrospective observational study of a wide and unselected series of patients diagnosed as primary RP from a single university hospital from January 2012 to December 2018. Patients were classified as primary RP after the presence of CTD at the onset was excluded. Results We studied 248 patients (210 women/38 men), with a mean age of 46.3±15.2 years (range 15-88). After a mean follow-up period of 57.2±21.6 months, 9 (3.6%) patients were diagnosed of a CTD. The remaining 239 (96.4%) patients continued classified as primary RP. The main pathologies observed after follow-up were: Scleroderma/Systemic sclerosis (n=3), Systemic Lupus Erythematosus (n=3), Rheumatoid arthritis (n=1), Sjogren’s syndrome (n=2). The main capillaroscopic patterns observed were: Normal (n=175), Unspecific (n=64), Scleroderma pattern (n=3) and suggestive of other rheumatic diseases pattern (n=6). All the patients who developed a CTD during follow-up showed changes at successive nailfold capillaroscopic examinations. 28 of 239 patients who remained primary RP showed minor changes at successive nailfold capillaroscopy. The main capillaroscopic changes detected on this group were: Presence of capillary tortuosity (n=6), presence of a decreased capillary density (2) and the presence of capillary bleeding (n=20). Antinuclear antibodies (ANA) were positive at he onset in 46 patients (16.9%) and after follow-up in 44 (17.7%) patients. Conclusion After a mean follow-up period next to 5 years, most of our primary RP patients remained free of CTD. A minority of our patients showed changes at nailfold capillaroscopy exam or positivity of ANA. Disclosure of Interests None declared

Background Raynaud’s phenomenon (RP) is a clinical expression of recurrent reversible vasospasm of small peripheral arteries and arterioles. It is a common pathology in clinical practice and is classified into two main categories – primary RP in the absence of an underlying disorder and secondary RP that is in the context of another disease. The differential diagnosis is of crucial importance for the practising rheumatologists because the patients with primary RP are with benign course while those with secondary RP require further differentiation and establishment of the precise diagnosis and treatment. Differentiation between primary and secondary RP is based on clinical features, laboratory including immunological investigations and capillaroscopic findings. Objectives The nailfold capillaroscopy is a key imaging tool for monitoring the RP patients because of the high predictive value of the abnormal capillaroscopic pattern for future development of connective tissue disease. Patients with primary RP have younger age at onset ( Methods Literature data on quantitative analysis of capillaroscopic parameters in primary RP are limited. Results In an own study that included 31 patients with primary RP patients, significantly wider arterial and venous capillary diameters as compared with healthy controls was found in 96.6% of cases. The mean arterial capillary diameter (0.018±0.004 mm) in primary RP patients was significantly larger in comparison healthy volunteers (0.012±0.001 mm, p Conclusions These capillaroscopic observations in primary RP patients suggest that the absence of an abnormal capillaroscopic pattern is diagnostic for primary RP but minor capillaroscopic dilation is a frequent microvascular feature in these patients. References [1] Lambova S, Muller-Ladner U. Capillaroscopic findings in primary fibromyalgia. Curr Rheumatol Rev2017. doi: 10.2174/1573397113666170607160854. [2] Bukhari M,Hollis S, Moore T, Jayson MI, Herrick AL. Quantitation of microcirculatory abnormalities in patients with primary Raynaud’s phenomenon and systemic sclerosis by videocapillaroscopy. Rheumatology (Oxford)2000;39(5):506–12. [3] Anderson ME, Allen PD, Moore T, Hillier V, Taylor CJ, Herrick AL. Computerized nailfold videocapillaroscopy – a new tool for assessment of Raynaud’s phenomenon. J Rheumatol2005;32:841–8. Disclosure of Interest None declared

Background Raynaud’s phenomenon (RP) is frequently associated with the presence of scleroderma or other connective tissue diseases (CTD). Identify the presence of secondary RP is important to perform an adequate therapeutic management and to achieve the early control of these patients. Nailfold capillaroscopy is safe, economic, and relatively easy to perform and has proven to be useful in identifying patients with secondary RP. Objectives To assess a long-term follow-up primary RP patient’s series. Methods Retrospective observational study of a wide and unselected series of patients diagnosed as primary RP from a single university hospital from January 2012 to August 2017. Patients were classified as primary RP after the presence of CTD at the onset was excluded. Results We studied 214 patients (85.98% were female), with a mean age of 47.6±16.7 years (range 15–88). After a mean follow-up period of 46.4±23.3 months, 8 patients were diagnosed of a CTD (2 Scleroderma/Systemic sclerosis, 3 Systemic Lupus Erythematosus, 1 Rheumatoid arthritis and 2 Sjogren’s syndrome). The remaining 206 patients continued classified as primary RP. The main capillaroscopic patterns observed were: Normal (n=157), unspecific (n=49), scleroderma pattern (n=2) and suggestive of other rheumatic diseases pattern (n=6). All patients who developed a CTD during the follow-up, showed changes in successive nailfold capillaroscopic examinations. 20 of 206 patients who remained primary RP showed minor changes at successive nailfold capillaroscopy. The main capillaroscopic changes detected on this group were: presence of capillary tortuosity (n=4), presence of a decreased capillary density2 and the presence of capillary bleeding (n=16). Antinuclear antibodies (ANA) were positive at the onset in 34 patients and after follow-up in 39 patients. Conclusions After a mean follow-up period next to four years, most of our primary RP patients remained free of CTD. A minority of our patients showed changes at nailfold capillaroscopy exam or positivity of ANA. Disclosure of Interest None declared

Background/aim: The pathogenesis of Raynaud's phenomenon (RP) has not yet been fully elucidated. RP is characterized by exaggerated cold-induced vasoconstriction. Urotensin II (UII) is a potent vasoconstrictor. The aim of the present study was to evaluate plasma UII levels in both primary RP and secondary RP associated with systemic sclerosis (SSc).Materials and methods: Fifteen patients with primary RP, 30 patients with RP secondary to SSc, and 30 healthy controls (HC) were included in the study. Raynaud condition scores (RCS) were determined in the primary RP and SSc groups. Modified Rodnan skin score (MRSS) was determined for the SSc patients. Plasma UII level was analyzed by the ELISA method. Results: When compared to the HC group, plasma UII level was lower in the secondary RP group, but not in the primary RP group. Plasma UII level was not directly related to RCS in either the primary or secondary RP group. Moreover, it was not correlated with MRSS in the secondary RP group.Conclusion: The results of the present study suggest that UII is not associated with primary RP. Its level was lower in the secondary RP (SSc) patients. Therefore, it can be concluded that decreased UII level is related to SSc instead of RP.

The aim of this study is to assess the prognostic value of major provisional criteria for the development of systemic sclerosis (SSc) in primary Raynaud’s phenomenon (RP) patients. We retrospectively studied the chart of 497 patients with primary RP in whom anticentromere (ACA) and antitopoisomerase I (ATA) antibodies tests and a capillary reading were available. Sensitivity, specificity, positive predictive value, negative predictive value, positive likelihood ratios (LHR+), negative likelihood ratios (LHR−), odds ratio (OR), and area under the receiver operating characteristics curve (AUC) of those criteria were assessed to predict the development of SSc. During the average follow-up of 2.3 ± 1.9 years, 159 (32 %) patients evolved to SSc, 245 (49.3 %) evolved to other connective tissue diseases, and 93 (18.7 %) patients did not progress. The SSc pattern predicted SSc satisfactorily (LHR+ 4.12, LHR− 0.07, OR 63, AUC 0.819; P

We hypothesized that mean platelet volume (MPV), a reliable marker of platelet activation, might be elevated in primary Raynaud's phenomenon (PRP) even if there was no thrombotic complication in our subjects. In this retrospective-cohort study, we examined the clinical value of MPV in 200 patients with PRP and 116 clinical controls, and measured MPV and platelet P-selectin (CD62P) in all study participants. We also evaluated the effect of age, gender, and disease duration on these platelet activation markers in PRP. MPV and CD62 positivities were significantly (p

Raynaud's phenomenon (RP) is defined as episodic ischemia of the extremities in response to cold. Although the structure of skin capillaries is normal in primary RP, some data suggest impairment of microvascular function. We aimed at testing whether digital skin blood flow was lower in RP than in controls while cooling locally. We further evaluated the contribution of sensory nerves in the response. We recruited 21 patients with primary RP and 20 healthy volunteers matched on age and gender. After a 10-min baseline at 33°C, skin temperature was cooled at 15 or 24°C during 30 min on the forearm and the finger while monitoring perfusion with a custom-design laser Doppler flowmetry probe. Perfusion was also assessed after topical anesthesia. Blood flow was expressed as cutaneous vascular conductance (CVC). Data were subsequently expressed as area above the curve (AAC0–30) of the percentage decrease from baseline CVC (%BL). CVC on the dorsum of the finger was lower in RP patients compared with controls at 15°C (AAC0–30 were 106,237.2 and 69,544.3%BL·s, respectively; P = 0.02) and at 24°C (AAC0–30 were 86,915 and 57,598%BL·s, respectively; P = 0.04) whereas we observed no significant difference on the finger pad and the forearm. Topical anesthesia increased CVC in patients with RP ( P = 0.05), whereas it did not affect reactivity in controls ( P = 0.86). Our study shows exaggerated skin microvascular vasoconstriction to local cooling on the dorsum of the finger in primary RP compared with controls. Part of this abnormal response in primary RP depends on sensitive nerves.

To investigate simultaneously skin perfusion and digital artery pulsatility of hands in patients with primary Raynaud's phenomenon (PRP) and systemic sclerosis (SSc).In 100 SSc patients, 92 PRP patients, and 80 healthy controls, perfusion and digital artery pulsatility of the hands were investigated by laser Doppler perfusion imaging (LDPI) and photoplethysmography (PPG), respectively.Both the mean value of mean perfusion and the mean value of sphygmic wave amplitude were lower (p0.0001) in the PRP group than in the SSc group, and also in the SSc group compared with the healthy controls. A positive correlation (r = 0.95) was found between the mean value of mean perfusion and the mean value of sphygmic wave amplitude. A homogeneous perfusion distribution pattern was present in 95% of the healthy controls, 93% of the PRP patients, and 4% of the SSc patients. PPG shows a homogeneous pattern in 95% of the healthy controls, 93% of the PRP patients, and 28% of the SSc patients. LDPI and PPG showed a positive concordance (p0.05) in homogeneous pattern evaluation. In the SSc patients, no correlation (r = 0.38) was observed between the mean value of sphygmic wave amplitude and the mean value of mean perfusion of each finger.LDPI and PPG can provide useful information in distinguishing patients with PRP and SSc, although nailfold videocapillaroscopy (NVC) is the best method for analysing microvascular damage in rheumatic diseases.

Pregnancy outcomes were assessed in 67 women who had well-defined primary Raynaud's phenomenon and in 30 controls, each of whom had been pregnant at least once. Twenty-seven patients had onset of Raynaud's phenomenon prior to any pregnancy, and 37 patients had onset of Raynaud's phenomenon after their last pregnancy. The frequency of miscarriage, stillbirth, and small full-term infants was similar in all 3 study groups. Among the 4 Raynaud's phenomenon patients who had second-trimester miscarriages, 2 of 3 who were tested had moderately positive levels of IgG or IgM anticardiolipin antibody. Premature births were significantly more common in pregnancies that occurred after the onset of Raynaud's phenomenon (24%) than in pregnancies that occurred prior to the onset of Raynaud's phenomenon or in pregnancies among control subjects (9% and 1%, respectively). In addition, the mean weight of the full-term babies of women in both Raynaud's phenomenon groups was significantly less than that of babies born to controls. There were no neonatal deaths or other serious adverse outcomes. These findings may represent yet another manifestation of systemic vasospasm in patients with primary Raynaud's phenomenon.

Ian Poxon, Jack Wilkinson, Tonia Moore, Joanne Manning, Mark Dickinson, Ariane Herrick and Andrea Murray Photon Science Institute, University of Manchester, Manchester, Research and Development, Directorate of Rheumatology, Salford Royal Hospital NHS Foundation Trust, Salford and Centre for Musculoskeletal Research, Manchester Academic Health Science Centre, University of Manchester, Manchester, UK

Resume Propos Des etudes recentes ont suggere que les patients porteurs d'une maladie de Raynaud etaient plus souvent porteurs d'une infection a Helicobacter pylori. Devant ces resultats nous avons effectue une etude prospective, comparative afin de tester la prevalence des infections a H. pylori au cours de la maladie de Raynaud. Methodes Quarante patients presentant une maladie de Raynaud ont ete inclus dans l'etude. Ils ont ete compares a 80 sujets sains apparies pour l'âge et le sexe. L'infection a H. pylori a ete recherchee en utilisant une serologie et un test respiratoire a l'uree. Resultats Chez les patients porteurs d'une maladie de Raynaud la prevalence de l'infection a H. pylori etait de 12,5 % en utilisant les deux methodes de recherche. Dans le groupe temoin la prevalence etait de 16,7 % par la methode serologique et de 18 % en utilisant le test a l'uree. Conclusion Les prevalences etant similaires dans le groupe maladie de Raynaud et le groupe temoin (p = 0,53 et 0,43 respectivement), il apparait que l'infection a H. pylori ne joue aucun role dans la genese de la maladie de Raynaud. Par ailleurs, les patients atteints de maladie de Raynaud souffraient significativement plus que les temoins de troubles digestifs (p

Results were as follows: 77 (62%) patients had cryofibrinogen in their blood plasma, 50 (40%) patients had precipitation of cryoglobulins, and 65 (52%) patients had an increase of α2globulin in the serum electrophoresis. Of the immunoglobulins, IgM was found increased in 42 patients (34%). Of the clotting time inhibitors, antithrombin III (AT III) had a lower activity in 50 (40%) patients as compared with that of normal subjects. Protein C, protein S, and α1antitrypsin levels were lower in 21 (16.8%), 13 (10.4%), and 10 (8%) of patients, respectively.Statistical analysis of the results showed that all the protein fractions except for γ-globulin presented a statistically significant difference as compared with those of the controls (P < 0.0001). Immunoglobulins IgG and IgA of the patients were significantly lower than those of the normal controls (P < 0.001). The C3 and C4 factors of the complement presented statistically significant lower values (P < 0.001 and P < 0.0001 respectively). Finally, AT III appeared in lower values than those of the normal controls, being statistically significant (P < 0.0001).These results suggest that patients with primary Raynaud's phenomenon present changes in the levels of certain hematologic parameters, a fact that is probably connected with etiology. Thus further study and assessment might contribute to the diagnosis, discovery of the subjective systemic etiology, and better therapeutic management of Raynaud's phenomenon.

Primary Raynaud9s phenomenon (PRP) is a common condition characterized by transient ischemia in the extremities in response to cold or emotions, which does not progress to digital ulcerations. The pathophysiology of PRP is multifactorial and complex, and while PRP is considered as a benign condition, it can be associated with significant disability during the cold season. The objective of this talk is to further clarify how a diagnosis of PRP can be performed, and to review the different pharmacological and non pharmacological treatments currently used in the management of RP, from their mechanism of action to the available evidence regarding their efficacy. We will also address the issue of drug-induced RP. Disclosure of Interest J.-L. Cracowski Grant/research support from: Pfizer; Bioprojet

Background Peripheral blood perfusion is reduced in patients with both primary (PRP) or secondary Raynaud9s phenomenon (SRP) (1–2). Objectives To investigate blood perfusion (BP) in different skin areas of hands in patients with PRP, SRP to systemic sclerosis (SSc), and healthy subjects (CNT). Methods 70 SSc patients (ACR/EULAR criteria) (3) (mean age 63±12 years, mean disease duration 6±5 years), 31 PRP patients (LeRoy criteria) (4) (mean age 48±18 years, mean Raynaud duration 3±2 years) and 68 CNT (mean age 59±19 years) were enrolled during winter time, after informed consent. BP was assessed by Laser speckle contrast analysis (LASCA) at the level of fingertips, periungual areas, dorsal and palmar aspect of 3rd finger bilaterally, dorsum and palm of both hands, and the average BP calculated as perfusion units (PU) (1). Nailfold videocapillaroscopy (NVC) was also performed to distinguish between PRP and SRP, and to detect the proper pattern of nailfold microangiopathy (“early”, “active” or “late”) in SSc patients (5). Patients were not taking vasodilator drug since at least two weeks. Results Both PRP and SSc patients showed a statistically significant lower BP than CNT at the level of fingertips (median 86, 88, 186 PU, respectively, p Conclusions By considering a small cohort of patients, BP of hand was found lower in PRP than in SSc patients with the “early” NVC pattern of microangiopathy. Also the gradients of perfusion between distal and proximal areas of hand were significantly lower in PRP than in SSc patients. The clinical value of this new early finding is matter of further analysis. References Ruaro B, et al. Ann Rheum Dis 2014;73:1181–5. Rosato E, et al. Rheumatology 2009;36:2257–63. van den Hoogen F, et al. Ann Rheum Dis 2013;72:1747–55. LeRoy EC, et al. Clin Exp Rheumatol.1992;10:485–8. Sulli A, et al. Arthritis Rheum. 2012;64:821–5. Disclosure of Interest None declared

Digital gangrene is not usually associated with primary Raynaud's phenomenon (RP). Its presence should therefore alert the healthcare provider to look for an alternative explanation. A 19-year-old female patient with primary RP developed digital gangrene following surgical management of acute paronychia. The possible mechanism in this patient appears to be the augmentation of the vasoconstrictive response due to the local infiltration of epinephrine mixed with lignocaine prior to the incision and drainage of her infected finger.

The magnesium (Mg) concentration was measured in erythrocytes of 30 women with pronounced primary Raynaud's phenomenon (PRP) and of 33 age-matched healthy control women. The measurements were made at three different times, six months apart. The mean Mg concentration in the PRP group and the control group differed signifi cantly at each measurement. The values for the two groups were, respectively 1.64 ±0.21 and 2.02 ±0.21 mmol/L (P

Raynaud’s phenomenon, episodic color change of the fingers usually in response to cold exposure, can be either primary (idiopathic: PRP) or secondary to an underlying disease or condition, for example, to a systemic sclerosis (SSc)-spectrum disorder. PRP is common and, although it can be painful and debilitating, is not generally considered a serious condition. This is because in patients with PRP, the episodes of digital ischemia are entirely reversible and do not progress to irreversible tissue injury with ulceration and scarring (as can occur in SSc).

Raynaud's phenomenon is a common clinical disorder consisting of recurrent, long-lasting and episodic vasospasm of the fingers and toes often associated with exposure to cold. In this article, we present a case of partial fingertip necrosis following digital surgical procedure in a patient with primary Raynaud's phenomenon.

This study was conducted to evaluate the diagnostic accuracy of power Doppler ultrasonography (PDU) for differentiating secondary from primary Raynaud’s phenomenon (RP), and also compared PDU with nailfold capillaroscopy (NFC) for the assessment of microvascularity in undifferentiated connective tissue disease (UCTD) patients with RP. Microvascularity in the nailfold and finger tip was evaluated using PDU with cold challenge, and the findings of PDU were classified according to the qualitative grading system before and after cold challenge. NFC was performed at the same day in all persons. The results of PDU were compared with the clinical, laboratory data, and the findings of NFC. The 14 UCTD patients with RP were included in our study. Seven patients were suspected of secondary RP in NFC examination, thus NFC yielded a correct classification into secondary RP in 50% of the UCTD patients. The PDU finding of pattern II, which is regarded as the finding of secondary RP, was observed in 12 UCTD patients with RP. Thus, PDU yielded a correct classification into secondary RP in 86% of UCTD patients. In conclusion, we confirmed that PDU has a higher correct classification rate than NFC for the diagnosing of secondary RP in UCTD patients. Our results suggest that PDU has better accuracy than NFC in differentiating secondary from primary RP, and PDU is more useful in assessing microvascular abnormalities in UCTD patients with RP.

Summary Objective This study investigated whether myofascial release techniques performed on upper body connective tissue could mitigate the frequency, duration or pain intensity associated with primary Raynaud's phenomenon. Methods Five treatments were administered over a 3-week treatment period on a 35-year-old female experiencing primary Raynaud's phenomenon for the past 12 years. A log was kept documenting frequency, duration and severity of pain. The myofascial work targeted the upper back, neck and arms according to hypothetical fascial meridian lines. Results Symptom duration was the one characteristic that showed improvement. After the first treatment, the duration of the subject's vasospastic episodes was reduced by almost half and continued to decrease throughout the 3 weeks of treatments. Neither the frequency or number of affected digits varied significantly from the pre-treatment weeks. Conclusions The results suggest that by releasing restricted fascia, myofascial techniques may influence the duration and severity of the vasospastic episodes experienced in primary Raynaud's phenomenon.

Background Laser speckle contrast analysis (LASCA) is a technique to assess blood perfusion in different body areas (1-2). Objectives To investigate peripheral blood perfusion (BP) by LASCA in different areas of hands in primary Raynaud9s phenomenon (PRP) patients and healthy subjects (CNT), looking for differences between the groups. Methods 31 PRP patients (LeRoy criteria) (3) (mean age 48±18 years, mean Raynaud duration 6±2 years) and 51 CNT (mean age 51±16 years) were enrolled, after informed consent. BP was bilaterally assessed by LASCA at the level of fingertips, periungual areas, dorsal and palmar surface of proximal phalanges, dorsum and palm of both hands, and the BP was calculated as perfusion units (PU) (1,2). All subjects stayed in a room at constant temperature (23°C) for 20 minutes before the analysis. PRP subjects discontinued treatment with oral vasodilators, if present, at least seven days before the examination. Statistical analysis was carried out by non parametric tests. Results PRP patients showed a statistically significant lower BP than CNT at the level of fingertips (median 86 and 181 PU, respectively, p Conclusions This study demonstrates that blood perfusion is reduced only in selected areas of the hands (where microcirculation is prevalent), sparing dorsal surface of phalanges and dorsum of hands (where macrocirculation is predominant). Therefore, present results are in agreement with the fact that macrocirculation is usually not involved in PRP (4). References Ruaro B, et al. Ann Rheum Dis 2014;73:1181-5. Sulli A, et al. Ann Rheum Dis 2014;73:2059-61. LeRoy EC, et al. Clin Exp Rheumatol.1992;10:485-8. Cutolo M et al. Nat Rev Rheumatol. 2010;6:578-87. Disclosure of Interest None declared

Background Primary Raynaud9s phenomenon (PRP) represents a benign condition observed in healthy subjects. It is usually due to an exaggerated response to the physiological cold-induced vasospasm [1]. Objectives To detect by nailfold videocapillaroscopy (NVC) the presence of age-related capillary morphological patterns in a large cohort of subjects with PRP. Methods 877 subjects affected by PRP (median age 44 years; median disease duration 3 years) performed NVC during their normal clinical screenings and they were divided into three groups (age 55 years, 254 patients). Two fields (3 mm) per finger of both hands were analyzed in the central area of the nailfold, and the qualitative score for several capillaroscopic parameters, usually observed in PRP patients, was evaluated (i.e. number of apical ectasias, irregular ectasias, efferent branch ectasias, microhaemorrhages, tortuosity, as well as subpapillary venous plexus visibility) [2]. The statistical analysis was performed by non-parametric tests. Results Patients with irregular ectasias, as well as patients with efferent branch ectasias, seem significantly younger than those without (median age 40 vs 48 years, and 36 vs 47, p 55 years (p 55 years (p 55 years (p Conclusions Aspecific abnormalities in nailfold microvasculature could be present in subjects with PRP with a different age distribution. The possible relationship with younger age and local trauma/damage, and/or as related to hypoxic conditions linked to the intensity/duration of the PRP are under further investigation. References LeRoy EC, Clin Exp Rheumatol 1992;10:485-8. Smith V,Pizzorni C. In Atlas of Capillaroscopy in Rheumatic Diseases. M. Cutolo Ed, Elsevier 2010. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3539

Background Raynaud’s phenomenon (RP) is a microcirculatory disorder characterised by deficient endothelial activity. Such endothelial dysfunction should be responsible for reduced release of NO, a potent vasodilatory agent. Since the cell function is dependent on energy production, it is though deficient endothelial activity could be due to the loss of energy production probably secondary to a low availability of Carnitine, an important cell metabolic carrier. Objectives To conduct a preliminary evaluation of the serum Carnitine concentrations in primary RP patients (pRP pts.) compared to age- and sex-matched controls. Methods After verbal consent, 17 consecutive pRP pts (16 female, mean age 32.1 ± 11.3 yrs) and ten control subjects (9 female, mean age 34 ± 11 yrs) were enrolled for this study. Patients had to have a RP history no longer than 5 years. After acclimatisation, each subject underwent a venous blood drawing from same vessel at baseline and after a 5-minute cold-water test. All blood samples were stored ?20°C. Then, Carnitine was measured in collected sera by enzymatic assay (SIGMA-TAU pharmaceuticals). T-test for paired data was used for statistical analysis. Results Table 1 showed the mean values of serum Carnitine concentrations in two studied groups. After test, serum Carnitine concentration increased in 12/17 pRP patients and lowered in the remainders. Contrary, in the control group circulating Carnitine increased significantly after provocative test. Conclusion Lower circulating Carnitine titres in our pRP pts could be associated with diminished availability of Carnitine in endothelial cells and consequently with their impaired activity. Although this study is preliminary, our results suggest Carnitine supplementation for promotion of endothelial activity in pRP pts may be worth investigating, particularly in RP subjects unresponsive to conventional treatments.

Objective. To assess nailfold capillary density and dimensions in patients with primary Raynaud's phenomenon (PRP), limited cutaneous systemic sclerosis (LSSc) and diffuse cutaneous SSc (DSSc), and healthy control subjects. Methods. Using the technique of nailfold video capillaroscopy, capillary density and dimensions were averaged from all visible capillaries in a 3 mm length of the nailfold from right and left ring fingers of each subject. Twenty healthy control subjects, 15 patients with PRP, 13 patients with DSSc and 21 patients with LSSc were examined. Intra‐observer and inter‐observer variability were calculated in 18 and 23 patients, respectively. Results. There were significant trends for capillary density to fall and for all dimensions to rise across the four groups (P

Three young children, aged 17 months, 26 months and 29 months, presented with primary Raynaud's phenomenon. One was treated with nifedipine. Raynaud's phenomenon is rare in children and as a result experience in its treatment limited. Previously reported cases of childhood Raynaud's are reviewed, and approaches to management discussed.

[No abstract available]