scholarly journals THU0357 NAILFOLD CAPILLARY MICROSCOPY HAS LIMITED PROGNOSTIC VALUE IN PREDICTING FUTURE DEVELOPMENT OF CONNECTIVE TISSUE DISEASE IN CHILDREN WITH RAYNAUD’S PHENOMENON.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 410.2-410
Author(s):  
G. Van der Kamp ◽  
D. J. Mulder ◽  
A. Van Roon ◽  
A. Van Roon ◽  
A. Van Gessel ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Prognostic Value ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Multivariate Logistic Regression Model ◽  
Multivariate Logistic Regression ◽  
Capillary Microscopy ◽  
Follow Up Study

Background:For adults with Raynaud’s phenomenon (RP), nailfold capillary microscopy (NCM) is established to be an effective method for differentiating between PRP and SRP (1,2). Although Raynaud’s phenomenon (RP) is very common in childhood, studies on diagnostic methods to differentiate between primary RP (PRP) and secondary RP (SRP) at a young age are scarce (3,4).Objectives:The general aim of this study was to determine the prognostic value of nailfold capillary microscopy (NCM) in addition to antinuclear antibodies (ANAs) for later development of connective tissue diseases (CTD) in children with RP.Methods:This was a case-control study, in which 83 patients diagnosed with RP and having undergone NCM in childhood were retrospectively included. Based on whether they were diagnosed with a connective tissue disease (CTD) during follow-up, they were classified as PRP or SRP. PRP and SRP patients were compared on demographics, NCM and ANA positivity. Variables associated with SRP were included in a multivariate logistic regression model. Predictive values were calculated for NCM, ANA positivity and the combination of NCM and ANA positivity.Results:At the time of the baseline NCM, the mean age of the RP patients was 15.4±2.3 years. Averagely 6.4±3.2 years after the baseline NCM, 65 of the 83 patients were classified as PRP and 18 as SRP. The most common CTDs were MCTD and undifferentiated CTD. ANA positivity was associated with SRP (p<0.001). Of the NCM parameters, only capillary loss was associated with SRP (p=0.01). Abnormal numbers of dilated capillaries, giant capillaries and haemorrhages were not significantly associated with SRP. In a multivariate logistic regression model, only ANA positivity was predictive for SRP (OR 11.19, CI 3.07-40.79). ANA alone had a sensitivity of 66.7% and a specificity of 85.9% for SRP. ANA combined with capillary loss had a sensitivity of 33.3% and a specificity of 96.8%.Conclusion:This study demonstrates that childhood RP is primary in most cases. Whereas RP in adulthood is most strongly associated with SSc, children with RP seem to be at risk of developing other CTDs with less apparent NCM abnormalities. Dilated capillaries, giant capillaries and haemorrhages on NCM are not associated with the spectrum of CTDs that children are at risk for, and do not differentiate between primary and secondary RP. Although capillary loss on NCM is associated with SRP, capillary loss may add little to the predictive value of serology. To clarify which NCM parameters are helpful for early detection of SSc-like CTDs, additional research is required.References:[1]Koenig M, Joyal F, Fritzler MJ, Roussin A, Abrahamowicz M, Boire G, et al. Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud’s phenomenon to systemic sclerosis: A twenty-year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis. Arthritis Rheum. 2008;58(12):3902–12.[2]Pavlov-Dolijanovic S, Damjanov NS, Stojanovic RM, Vujasinovic Stupar NZ, Stanisavljevic DM. Scleroderma pattern of nailfold capillary changes as predictive value for the development of a connective tissue disease: a follow-up study of 3,029 patients with primary Raynaud’s phenomenon. Rheumatol Int. 2012;32(10):3039–45.[3]Jones GT, Herrick AL, Woodham SE, Baildam EM, Macfarlane GJ, Silman AJ. Occurrence of Raynaud’s phenomenon in children ages 12-15 years: Prevalence and association with other common symptoms. Arthritis Rheum. 2003;48(12):3518–21.[4]Pavlov-Dolijanović S, Damjanov N, Ostojić P, Sušić G, Stojanović R, Gacić D, et al. The prognostic value of nailfold capillary changes for the development of connective tissue disease in children and adolescents with primary Raynaud phenomenon: A follow-up study of 250 patients. Pediatr Dermatol. 2006;23(5):437–42.Disclosure of Interests:None declared

scholarly journals AB0576 INCIDENCE AND CLINICAL MANIFESTATIONS OF RAYNAUD’S PHENOMENON IN RHEUMATIC DISEASES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1584.1-1585
Author(s):  
I. Gaisin ◽  
Z. Bagautdinova ◽  
R. Valeeva ◽  
N. Maximov ◽  
O. Desinova ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Rheumatic Diseases ◽  
Single Phase ◽  
Raynaud’S Phenomenon ◽  
Colour Change ◽  
Raynaud's Phenomenon ◽  
Phase Changes ◽  
Three Phase

Background:Systemic sclerosis (SSc) is a connective tissue disease (CTD) most frequently associated with Raynaud’s phenomenon – RP (96%), followed by mixed CTD (MCTD) (86%), systemic lupus erythematosus – SLE (31%), undifferentiated CTD (30%), rheumatoid arthritis – RA (22%) and Sjogren’s disease – SD (13%)1. RP can manifest as a classical triple-colour change with pallor (ischaemic phase) followed by cyanosis (deoxygenation) and erythema (reperfusion)2,1. However, this triple-colour change only occurs in 19% of cases2,3. Majority of patients report an episodic double-colour change, consisting of pallor and cyanosis, pallor and erythema or cyanosis and erythema2. In a 4.8-year follow-up, 37.2% of RP patients developed rheumatic diseases (RD), 8.1% had other causes, in 54,7% RP remained primary4.Objectives:To study the incidence and manifestations of secondary RP in RD.Methods:A questionnaire survey conducted in 230 patients with RD.Results:RP was detected in 45.6% of RD patients (n=105), 54.4% of patients with RD had no RP (n=125). RP was 4 times more frequent in females than in males (F:M 4:1). In RP group, 87 patients (82%) had autoimmune RD: SSc (55.2%), SLE (17.1%), RA (6%),dermatomyositis (3.8%), cross syndrome (3.8%), MCTD (1.9%), SD (0.9%).Only 84% of RP patients had positive answers to all three questions that characterizeRP (1. Is there an unusual sensitivity of fingers to cold? 2. Do fingers change colorwhen exposed to cold? 3. Do they turn white and/or bluish?). Biphasic color changes (whitening-blueness; whitening-redness; blue-redness) were observed in 33 (31.4%) patients with RP, three-phase changes – in 32 patients (30.5%). Blueness of fingers to cold was more frequent in SLE than in SSc (p=0.027).Redness of fingers to cold occurred more often in cross syndrome, MCTD, SD, RA, vasculitis than in SSc (p<0.001) and in vasculitis than in SLE (p=0.035). In SSc patients, whitening of fingers to cold was more common than redness (p=0.037) and two-/three-phase changes of fingers color in the cold were more frequent than single-phase changes (p<0.001).The frequency of RP attacks was detected more than once a day in 44 (42%) patients. In 73% of cases, RP did not show signs of deep digital ischemia. Digital ulcers (active) were observed in 13 (12.3%) patients, fractures in a finger area – 23 (21.9%), digital scars – 15 (14.2%), phalange amputations – 7 (6.6%).Conclusion:Patients with RD and secondary RP most often have SSC (55%), less often – SLE (17%), RA (6%), DM (3%). In SSc and SLE patients, Raynaud’s reddening of fingers to cold is less common than in other RD. In SSc, two-/three-phase changes of fingers color in the cold are more frequent than single-phase changes. In SLE, fingers turn blue in the cold more often than in SSc.References:[1]Prete M, Fatone MC, Favoino E, Perosa F. Raynaud’s phenomenon: from molecular pathogenesis to therapy.Autoimmun Rev2014;13:655–67.[2]Linnemann B, Erbe M. Raynaud’s phenomenon – assessment and differential diagnoses.Vasa2015;44:166–77.[3]Heidrich H, Helmis J, Fahrig C, Hovelmann R, Martini N. Clinical characteristics of primary, secondary and suspected secondary Raynaud’s syndrome and diagnostic transition in the long-term follow-up. A retrospective study in 900 patients.Vasa2008;37 (Suppl. 73):3–25.[4]Pavlov–Dolijanovic S, Damjanov NS, VujasinovicStupar NZ, Radunovic GL, Stojanovic RM, Babic D. Late appearance and exacerbation of primary Raynaud’s phenomenon attacks can predict future development of connective tissue disease: a retrospective chart review of 3035 patients.RheumatolInt2013;33:921–6.Acknowledgments:Professor LP. Anan’eva, Professor RT. AlekperovDisclosure of Interests:Ilshat Gaisin Speakers bureau: Boehringer Ingelheim, KRKA, Berlin-Chemie Menarini, Sanofi, Zukhra Bagautdinova: None declared, Rosa Valeeva: None declared, Nikolay Maximov Speakers bureau: Pfizer, KRKA, Oxana Desinova: None declared, Rushana Shayakhmetova: None declared, Irina Sabelnikova: None declared, Anna Tukmacheva: None declared, Larisa Gibadullina: None declared, Natalya Burlaeva: None declared, Elena Agareva: None declared, Yulia Ochkurova: None declared, Tatyana Bragina: None declared, Ksenia Alexandrova: None declared, Elvira Reutova: None declared

scholarly journals Impaired endothelial function in patients with undifferentiated connective tissue disease: a follow-up study

Rheumatology ◽  
2014 ◽  
Vol 53 (11) ◽  
pp. 2035-2043 ◽  
Author(s):  
R. Laczik ◽  
P. Soltesz ◽  
P. Szodoray ◽  
Z. Szekanecz ◽  
G. Kerekes ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Endothelial Function ◽  
Connective Tissue Disease ◽  
Undifferentiated Connective Tissue Disease ◽  
Follow Up Study

Connective tissue disease

2019 ◽  
pp. 281-326
Author(s):  
Gavin Spickett
Keyword(s):  
Rheumatoid Arthritis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Mixed Connective Tissue Disease ◽  
Raynaud’S Phenomenon ◽  
Connective Tissue Diseases ◽  
Raynaud's Phenomenon ◽  
Overlap Syndromes

This chapter covers the presentation, immunogenetics, immunopathology, diagnosis, treatment, and testing for a range of connective tissue diseases. It covers a range of rheumatic disorders, from rheumatoid arthritis to Raynaud’s phenomenon, and also covers the undifferentiated diseases, overlap syndromes, and mixed connective tissue disease.

Overlap/undifferentiated syndromes

2013 ◽  
pp. 1069-1078 ◽  
Author(s):  
Ariane Herrick
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Clinical Manifestations ◽  
Raynaud's Phenomenon ◽  
Overlap Syndromes ◽  
Clear Cut ◽  
Positive Treatment

Undifferentiated connective tissue disease (UCTD) and overlap syndromes both form part of the broad spectrum of connective tissue disease. They are difficult to define, as the boundaries between them and specific diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and myositis are often not clear-cut. This chapter gives a broad overview of diagnosis, clinical features, outcomes, and management. Patients with UCTD have clinical and/or serological features of connective tissue disease but do not fulfil the criteria for any one defined disease. Raynaud’s phenomenon and puffy fingers are often the presenting features but there are many possible others, including arthralgia, sicca symptoms, and breathlessness due to pulmonary fibrosis, usually in the context of a positive anti-nuclear antibody (ANA). A proportion of patients evolve into a defined connective tissue disease: in those who do, this is generally within 5 years of onset. Treatment is dependent upon the clinical features: for examplem vasodilators for Raynaud’s phenomenon, or hydroxychloroquine for arthralgia/arthritis. Patients with overlap syndromes have features of more than one defined connective tissue disease. Overlap syndromes are therefore highly heterogeneous as many combinations of clinical and serological features can occur. Mixed connective tissue disease (MCTD) is the overlap syndrome that has been most described and includes overlapping features of SLE, SSc, and myositis in patients who are anti-U1 ribonucleoprotein (RNP) antibody positive. Treatment is of the specific clinical manifestations. Patients with overlap syndromes should be kept under regular review to allow early identification of internal organ involvement.

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