Systemic involvement and immunologic findings in patients presenting with Raynaud's phenomenon

doi:10.1016/0002-9343(80)90417-9

The American Journal of Medicine

Volume 69, Issue 5, November 1980, Pages 675-680

https://doi.org/10.1016/0002-9343(80)90417-9 Get rights and content

Abstract

Systemic involvement and spectrum of autoantibodies were evaluated in 91 patients presenting with Raynaud's phenomenon. Decreased pulmonary diffusing capacity was observed in 23 percent, esophageal hypomotility in 14 percent and renal involvement in 5 percent of the patients, all without clinical symptoms. Arthralgia or a history of arthritis was present in 27 percent and skin abnormalities in 30 percent. Extent of systemic involvement was correlated with the severity of Raynaud's phenomenon, as measured by photoelectric plethysmography (r = 0.38; p < 0.01). In addition, both the variety of different autoantibodies in the serum of individual patients and the titer of antinuclear antibodies were positively correlated with the number of affected organ systems (r = 0.63; p < 0.01 and r = 0.65; p < 0.01, respectively). Raynaud's phenomenon is an important clinical sign of asymptomatic systemic disease. Measurements of its severity and serologic parameters are helpful in predicting the extent of systemic involvement.

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Upper Extremity Ischemia: Small Artery Occlusive Disease
2009, Comprehensive Vascular and Endovascular Surgery: Second Edition
Digital flow exploration by color Doppler ultrasound in patients with Raynaud's disease or systemic sclerosis
2004, Journal des Maladies Vasculaires
Le phénomène de Raynaud (PR) est un acrosyndrome vasculaire paroxystique fréquent, le plus souvent bénin, il s’agit alors de la maladie de Raynaud (MR). Il peut révéler une connectivite, en particulier la sclérodermie systémique (SS). Nous avons cherché à analyser, dans cette étude, la microcirculation pulpaire chez ces 2 types de patients, MR et SS, en utilisant l’écho-doppler digital.
L’examen écho-doppler était réalisé grâce à l’utilisation d’une sonde matricielle haute fréquence (VFX 13-5 Siemens) qui permet une meilleure résolution. Ainsi, nous avons pu mesurer les diamètres des artères digitales latérale et médiale, puis analyser la microcirculation pulpaire de façon quantitative et qualitative. Les mesures étaient effectuées à 25°C, 45 °C, 11 °C et après 5 minutes de récupération. Trente-trois patients ont été inclus : 14 présentant une maladie de Raynaud, 19 atteints de sclérodermie systémique répondant aux critères de l’ACR (American College of Rheumatology).
Concernant les diamètres des artères digitales, les mesures étaient significativement plus élevées chez les patients présentant une maladie de Raynaud que chez ceux atteints de sclérodermie systémique. De la même façon, la microcirculation pulpaire était significativement plus riche en cas de maladie de Raynaud que de sclérodermie.
L’écho-doppler digital permet donc de montrer des différences significatives morphologiques et hémodynamiques entre MR et SS.
Raynaud's phenomenon (RP) is a frequent vascular paroxysmal syndrome of the extremities. Generally benign, the condition is called Raynaud's disease (RD), which may reveal a connective tissue disease, particularly systemic sclerosis (SS). We evaluated digital blood flow in patients with RD and SS using color Doppler ultrasound.
Ultrasound examination was performed with a newly developed multi-D linear array transducer (VFX 13-5 Siemens), which allows better resolution. We first measured the diameters of the digital arteries (appendix 1) then epidermal, dermal, and hypodermal thickness for each patient (appendix 3) and performed a qualitative and quantitative analysis of pulpar microcirculation (appendix 4 & 5). All measures were made at 25°C, 45°C, 11°C and after recovery. Thirty-three patients were included: 14 with primary RD and 19 with SS as assessed by American College of Rheumatology criteria.
The diameters of the digital arteries showed significant discrepancies allowed to distinguish primary RP from SS. At 11°C, diameters were 0.6 ± 0.2 mm for primary RP versus 0.2 ± 0.3 mm for SS on lateral digital arteries (LDA) [p = 0.005]; 0.7 ± 0.2 mm for primary RP versus 0.4 ± 0.3 mm for SS on medial digital arteries (MDA) [p = 0.004]. After recovery, these diameters were respectively 1 ± 0.2 mm versus 0.5 ± 0.4 mm for LDA [p = 0.000] and 1 ± 0.2 mm versus 0.6 ± 0.3 mm for MDA [p = 0.000] (tables 1, 2, 3, 4). Similarly, pulpar vascularization was significantly higher in primary RP than in SS (tables 6, 7, 8). No difference was found in skin thickness nor in the epidermal aspect between the two groups (table 5).
Color Doppler ultrasound shows morphological and dynamic differences between RD and SS.
Antiendothelial cell antibodies: Useful markers of systemic sclerosis
1997, American Journal of Medicine
BACKGROUND: Systemic sclerosis (SS) encompasses a wide spectrum of clinical presentations. Antiendothelial cell antibodies (AECA) in patients with primary Raynaud's phenomenon (PRP), limited SS (lSSc), or diffuse SS (dSSc) may help to determine the long-term prognosis of the disease.
METHODS: Twenty-seven normal controls, 13 patients with PRP, 36 with lSSc, and 31 with dSSc were included in the study. Sera were examined for the presence of AECA, using a cellular enzyme-linked immunosorbent assay (ELISA). Angiotensin-converting enzyme (ACE) activity, plasma von Willebrand factor antigen (vWfAg), and thrombomodulin (Tm) concentrations were also evaluated. The medical records of 50 of the lSSc and dSSc patients were reviewed and the organ system involvement noted.
RESULTS: Antiendothelial cell antibodies were present in 3 patients with PRP, 16 patients with lSSc, and 26 patients with dSSc. These autoantibodies were mainly of the IgG isotype. There was no difference in ACE activity between patients and controls. In contrast, vWfAg and Tm concentrations were higher in patients with PRP relative to controls, and higher in patients with lSSc compared with those with PRP. The presence of AECA was associated with digital scars and ulcers (P < 0.004 and P < 0.003, respectively), severe RP (P < 0.01), grade 3 tortuosity of vessels (P < 0.0004), and lung involvement (P < 0.02).
CONCLUSION: The significant trend for AECA to increase with disease severity across the three groups of patients studied suggests that the AECA test can identify subsets of SSc with differing prognoses.
Diagnosis and long-term clinical outcome in patients diagnosed with hand ischemia
1995, Journal of Vascular Surgery
Purpose: The long-term clinical outcome of patients diagnosed with digital artery obstruction and symptomatic hand ischemia is largely unknown. Our long-term experience with the diagnosis of symptomatic digital artery obstruction and the long-term natural history of this condition forms the basis for this report.
Methods: From 1971 to 1985, 44 patients with symptomatic hand ischemia and palmar or digital arterial obstruction underwent arteriography and digital photoplethysmography (PPG). Patients were grouped according to severity of hand ischemia symptoms, including ulceration and digital amputation and the presence of a connective tissue disease (CTD). Arteriography was compared with PPG by creating an objective severity scale (digital obstruction index [DOI]).
Results: Average follow-up was 15.2 years (range 10 to 22 years). Initially 21 patients (48%) had moderate symptoms, and 23 patients (52%) had severe symptoms of hand ischemia. Follow-up symptoms in 28 patients improved (13 of 26 with CTD, 15 of 18 without CTD), in 15 patients (12 of 26 with CTD, 3 of 18 without CTD) remained unchanged, and in only 1 patient (1 of 26 with CTD) worsened. Seventeen (65%) patients with CTD (n = 26) had development of one or more ulcers, and six (24%) underwent one or more digital amputations. Four (22%) patients without CTD (n = 18) had finger ulceration (p < 0.012 compared with patients with CTD), and one patient (6%) underwent subsequent digital amputation (p = NS). The arteriography-DOI and PPG-DOI were equally accurate in determining severity of finger ischemia as manifested by severity of symptoms or ulcer development.
Conclusions: The favorable long-term prognosis of symptomatic finger artery occlusion described herein mandates the avoidance of premature finger amputation. Patients with CTD fare worse, but even in this group tissue loss is modest. Finger PPG is as accurate as arteriography for determining severity of hand ischemia. (J VASC SURG 1995;22:361-9.)
Diagnosis and treatment of vasospastic disorders
1995, Primary Care Update for Ob/Gyns
Vasospastic disorders of the extremities commonly present in women of reproductive age. For this reason, the primary care obstetrician/gynecologist must be knowledgeable with regard to the classification, diagnosis, and treatment of these disorders. A thorough understanding of the disease processes will allow the primary care physician to manage the majority of patients with vasospastic disorders and refer unusual or refractory cases to vascular medicine specialists.
Raynaud's Syndrome
1994, Annals of Vascular Surgery

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¹: From the Department of Clinical Immunology and Cardiology, University Hospital, Groningen, The Netherlands.

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Clinical studySystemic involvement and immunologic findings in patients presenting with Raynaud's phenomenon

Abstract

Am J Med

Lancet

Lancet

Am J Med

Pathophysiology of Raynaud's Disease

Arch Intern Med

Raynaud's phenomenon. Photoelectric plethysmography of the fingers of persons with and without Raynaud's phenomenon during cooling and warming up

Acta Med Scand

The mechanisms of Raynaud's phenomenon. I

Med J Aust

Survival in scleroderma

Ann Rheum Dis

Systemic lupus erythematosus in the community

Arch Intern Med

Lupus erythematosus

Clinical study
Systemic involvement and immunologic findings in patients presenting with Raynaud's phenomenon