Raynaud's phenomenon is a symptom complex manifested as intermittent fingertip ischemia caused by cold or other sympathetic drivers. Secondary Raynaud's phenomenon is often more severe and could even lead to finger ulceration, making it particularly complicated to treat. We describe a case of severe Raynaud's phenomenon secondary to subclinical hypothyroidism lasting for more than 6 hours in a 65-year-old woman. The patient was also diagnosed with hypothyroidism, epilepsy, and secondary soft tissue infection of the right middle and ring fingers. After careful multidisciplinary consultation and discussion, the patient received vasodilation, anticoagulation, thyroxine supplementation, stellate ganglion block, hyperbaric oxygen therapy and debridement. The patient responded well to the medication, avoiding amputation or obviously dysfunction. Multidisciplinary team gathering the doctors from different departments proposes appropriate strategies for patients with severe Raynaud's phenomenon and could improve the prognosis and satisfaction of patient effectively.
Female ; Humans ; Aged ; Hypothyroidism/complications* ; Raynaud Disease/diagnosis*

OBJECTIVE: To investigate the clinical and immunological characteristics of overlap myositis (OM) patients. METHODS: The data of 368 patients with idiopathic inflammatory myopathies (IIMs) admitted to Peking University People's Hospital from January 2004 to August 2020 were analyzed retrospectively, including demographic characteristics, clinical characteristics (including fever, Gottron' s sign/papules, Heliotrope rash, V-sign, Shawl sign, Mechanic' s hands, skin ulceration, periungual erythema, subcutaneous calcinosis, dysphagia, myalgia, myasthenia, arthritis, Raynaud' s phenomenon, interstitial lung disease, pulmonary hypertension and myocardial involvement), laboratory characteristics, immunological characteristics [including antinuclear antibodies, rheumatoid factors, myositis-associated autoantibodies (MAAs) and myositis-specific autoantibodies (MSAs)] and survival. The clinical and immunological characteristics and prognostic differences of OM and non-OM were compared. The Kaplan-Meier and Log Rank methods were used to analyze the survival. RESULTS: A total of 368 patients were included. 23.9% (88/368) of IIMs patients were OM patients. Among the 88 OM patients, 85.2% (75/88) of them were female, and the median interval between disease onset and diagnosis was 13.5 months. The incidence of overlapped connective tissue diseases in the OM patients was dermatomyositis (DM) in 60.2%, polymyositis (PM) in 3.4%, immune-mediated necrotizing myopathy (IMNM) in 2.3% and anti-synthetase syndrome (ASS) in 34.1%. Compared with the non-OM patients, the proportion of the females in the OM patients was higher (85.2% vs. 72.1%, P=0.016), the OM patients had longer disease duration [13.5(4.5, 48.0) months vs. 4.0(2.0, 12.0) months, P < 0.001]. As for clinical characteristics, compared with the non-OM patients, the incidence of V-sign (25.0% vs. 44.6%, P=0.001) and periungual erythema (8.0% vs. 19.6%, P=0.013) were lower; the incidence of Raynaud's phenomenon (14.8% vs. 1.8%, P < 0.001), interstitial pneumonia (88.6% vs. 72.1%, P=0.001), pulmonary hypertension (22.7% vs. 7.5%, P < 0.001) and myocardial involvement (18.2% vs. 9.3%, P=0.033) were higher. As for immunological characteristics, compared with the non-OM patients, the incidence of elevated aspartate aminotransferase (AST) (31.8% vs. 45.0%, P=0.035) was lower and elevated C-reactive protein (CRP) (58.0% vs. 44.6%, P=0.037) was higher; the positive rates of antinuclear antibodies (ANA) (85.1% vs. 63.4%, P=0.001) and rheumatoid factors (RF) (40.2% vs. 17.8%, P < 0.001) and anti-Ro-52 (71.6% vs. 56.1%, P=0.038) in serum were higher. There was no significant difference in the survival between the OM patients and non-OM patients. CONCLUSION Pulmonary hypertension and myocardial involvement were frequently observed in OM.
Autoantibodies ; Dermatomyositis/epidemiology* ; Female ; Humans ; Myositis/epidemiology* ; Raynaud Disease ; Retrospective Studies

OBJECTIVE: To evaluate the correlation between nailfold capillaroscopic (NC) findings and the presence of interstitial lung disease (ILD) in systemic sclerosis (SSc) patients. METHODS: We retrospectively involved 71 SSc patients, 45 patients with ILD. NC was performed in all the patients according to the standard method. The NC findings were semi-quantitatively scored, including enlarged and giant capillaries, hemorrhages, loss of capillaries, avascular areas, ramified/bushy capillaries and disorganization of the vascular array. The demographic and clinical data collected were gender, age, presence/absence of Raynaud phenomenon (RP), duration of RP, serological acute phase reactants and antibodies, presence of ILD (also evaluated the CT score for ILD) and pulmonary function parameters. RESULTS: Among the 71 patients, the frequency of the women was 91.5%, the mean age was (52.59±12.77) years, and disease duration was (3.00±6.00) years. NC changes of the scleroderma pattern were observed in 90.1% patients. There were 45 patients with ILD and 26 patients without ILD. The patients with ILD had significantly higher loss of capillaries score [0.50 (1.03) vs. 0.00 (0.43), P=0.003], avascular area score [0.75 (1.24) vs. 0.25 (0.83), P=0.006] and ramified/bushy capillaries score [0.33 (0.88) vs. 0.13 (0.25), P=0.006] compared with those without ILD. Moreover, ramified/bushy capillaries score together with diffused SSc were independent risk factors for the presence of ILD. And the score of giant capillaries were significantly lower in the patients with more severe ILD group [0.25 (0.94) vs. 0.00 (0.28), for the mild and severe ILD groups respectively, P=0.019]. There was no statistically significant difference between the two groups with respect to the scores of enlarged capillaries, hemorrhages, or disorganization of the vascular array. CONCLUSION Capillary deletion and severe deformity in NC were associated with the presence of ILD in SSc patients. And patients with less giant capillaries had more severe ILD involvement. These indicated that NC maybe a useful tool to evaluate ILD in SSc.
Adult ; Aged ; Capillaries ; Female ; Hemorrhage ; Humans ; Lung Diseases, Interstitial/complications* ; Male ; Microscopic Angioscopy ; Middle Aged ; Nail Diseases/complications* ; Nails ; Raynaud Disease ; Scleroderma, Systemic/complications*

BACKGROUND: Systemic sclerosis (SSc) is a rare, connective tissue  disease  with  multisystem involvement.This is due to immunological processes,vascular endothelial cell injury  and extensive activation of fibrolast that commonly affects the skin and other internal organs such as the esophagus, lungs, heart, and kidneys. SSc has one of the highest mortality among  the  autoimmune  rheumatic  diseases,  hence  the  emphasis  on  the  early  recognition  and  management  to  prevent significant progression of the disease.
CASE: A 22-year-old  female  presented  with  a  one-year history of multiple hard and hypopigmented patches on the face, neck, trunk and upper extremities. Further examination revealed mask-like facies, microstomia, frenulum sclerosis, Raynaud's phenomenon, pitted scars on the digital pulp of hands  and  sclerodactyly.Baseline  blood  chemistry,chest radiograph and electrocardiography  were  all  negative  for systemic involvement. Autoantibodies were positive for dsDNA, SS-A/Ro and Scl-70. Skin biopsy revealed sclerosing dermatitis, which was consistent with SSc.
OUTCOME: The  patient  was  initially  started  with  oral prednisone 0.5 mg/kg/day and was increased to 0.75 mg/kg/day for eight weeks. Prednisone was slowly tapered to 5.0 mg/day and methotrexate 15.0 mg/week was included in  the  management for eight weeks which resulted in decreased joint pains, halted the progression of skin induration, decreased in pruritus and palmar edema.
CONCLUSION: The characteristic dermatological findings of SSc are not only important signs to dermatologists, but these serves as diagnostic clues for clinicians from other disciplines as well. In our case, the presence of the autoantibody Scl-70 indicated the potential risk of pulmonary fibrosis and pulmonary arterial hypertension that accounts with high mortality.Hence,physicians should be aware of the possible risk of organ damage,even when asymptomatic because there is a high risk of disease progression. The importance of early recognition and a multidisciplinary approach lead to the good outcome in this case.

Human ; Female ; Adult ; Autoantibodies ; Prednisone ; Methotrexate ; Cicatrix ; Microstomia ; Sclerosis ; Raynaud Disease ; Pulmonary Fibrosis ; Hypertension, Pulmonary ; Edema ; Rheumatic Diseases ; Scleroderma, Systemic ; Scleroderma, Diffuse

Antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibody to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, mechanic's hand-like cutaneous involvement, Raynaud phenomenon, and polyarthritis. Lung disease is the presenting feature in 50% of the cases. We report a case of a 60-year-old female with acute respiratory distress syndrome (ARDS), which later proved to be an unexpected and initial manifestation of anti-Jo-1 antibody-positive antisynthetase syndrome. The present case showed resolution of ARDS after treatment with high-dose corticosteroids. Given that steroids are not greatly beneficial in the treatment of ARDS, it is likely that the improvement of the respiratory symptoms in this patient also resulted from the prompt suppression of the inflammatory systemic response by corticosteroids.
Adrenal Cortex Hormones ; Arthritis ; Dermatomyositis ; Female ; Fever ; Humans ; Immunoglobulins ; Ligases ; Lung Diseases ; Lung Diseases, Interstitial ; Middle Aged ; Myositis ; Polymyositis ; Raynaud Disease ; Respiratory Distress Syndrome, Adult* ; RNA ; Steroids

OBJECTIVES: To investigate the clinical findings upon initial diagnosis and extraglandular manifestations in Korean patients with primary Sjogren's syndrome (pSS). METHODS: We collected clinical and laboratory data from 238 pSS patients enrolled at Seoul National University Hospital, Seoul National University Bundang Hospital and Seoul Medical Center from March 2011 to December 2014. All patients met the American-European Consensus Group criteria for pSS. RESULTS: Upon initial diagnosis, sicca symptoms (xerophthalmia or xerostomia) as the chief complaint were only observed in 129 (54.2%) pSS patients, while extraglandular manifestation was more common as the chief complaint in male patients or those with younger age (<40 years) than female or older patients (both p<0.05). Extraglandular manifestations were found in 178 (74.8%) patients, with musculoskeletal manifestations being most common (53.8%). Peripheral neuropathy in pSS patients was associated with Raynaud phenomenon and elevated serum total immunoglobulin G (IgG) levels (both p<0.05). Serum beta2-microglobulin (beta2-M) levels were significantly correlated with European League against Rheumatism (EULAR) Sjogren's syndrome disease activity index, erythrocyte sedimentation rate and serum total IgG (all p<0.001), and were higher in patients with extraglandular manifestations than those without (p<0.05). Serum C3 levels were decreased in patients with extraglandular manifestation, compared to those without (p<0.05). Malignant lymphoma was found in Korean pSS patients (1.7%) and associated with elevated serum beta2-M levels (p<0.0001). CONCLUSION: Extraglandular manifestations were common in pSS patients and may be a diagnostic tool for male or younger pSS patients. Serum beta2-M levels can be useful markers for monitoring pSS patients.
beta 2-Microglobulin ; Consensus ; Diagnosis ; Erythrocyte Indices ; Female ; Humans ; Immunoglobulin G ; Lymphoma ; Male ; Peripheral Nervous System Diseases ; Raynaud Disease ; Rheumatic Diseases ; Seoul ; Sjogren's Syndrome*

BACKGROUND/AIMS: We investigated whether transthoracic echocardiography-suspected pulmonary hypertension (PH) affects survival in systemic lupus erythematosus (SLE) patients and examined factors associated with PH occurrence and survival. METHODS: This retrospective single-center study included 154 Korean SLE patients fulfilling the American College of Rheumatology criteria (January 1995 to June 2013). Student t test, Mann-Whitney U test, Kaplan-Meier curves, and log-rank tests were used for comparisons. RESULTS: A total of 35 SLE patients with PH (SLE/PH+) and 119 without PH (SLE/PH-) were analyzed. Higher percentages of interstitial lung disease, Raynaud's phenomenon (RP), World Health Organization functional classification III/IV, and cardiomegaly were found in SLE/PH+ compared to SLE/PH-. Furthermore, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index was significantly higher in SLE/PH+ (2.46 +/- 1.245 vs. 1.00 +/- 1.235), whereas survival rates were significantly higher in SLE/PH- in log-rank tests (p = 0.001). In multivariate analysis, the adjusted mortality hazard ratio (HR) for SLE/PH+ patients was 3.10. Subgroup analysis demonstrated a higher percentage of lupus nephritis in the SLE/PH+ patients who died (p = 0.039) and low complement-3 levels (p = 0.007). In univariate analysis, the mortality HR for SLE/PH+ patients with lupus nephritis was 4.62, whereas the presence of RP decreased the mortality risk in multivariate analysis; adjusted HR, 0.10. CONCLUSIONS: PH is an independent factor predicting survival in SLE patients. The presence of lupus nephritis resulted in an increased trend for mortality, whereas coexistence of RP was associated with a better survival prognosis in SLE/PH+ patients.
Adolescent ; Adult ; Cardiomegaly/diagnosis/epidemiology ; Chi-Square Distribution ; Female ; Humans ; Hypertension, Pulmonary/diagnosis/*mortality ; Kaplan-Meier Estimate ; Lung Diseases, Interstitial/diagnosis/mortality ; Lupus Erythematosus, Systemic/diagnosis/*mortality ; Lupus Nephritis/diagnosis/mortality ; Male ; Multivariate Analysis ; Prognosis ; Proportional Hazards Models ; Raynaud Disease/diagnosis/epidemiology ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Young Adult

BACKGROUND: Surgical scars on the palmar surface of the hand may lead to functional and also aesthetic and psychological consequences. The objective of this study was to introduce a new incision technique for periarterial sympathectomy of the hand and to compare the results of the new two-step incision technique with those of a Koman incision by using an objective questionnaire. METHODS: A total of 40 patients (17 men and 23 women) with intractable Raynaud's disease or syndrome underwent surgery in our hospital, conducted by a single surgeon, between January 2008 and January 2013. Patients who had undergone extended sympathectomy or vessel graft were excluded. Clinical evaluation of postoperative scars was performed in both groups one year after surgery using the patient and observer scar assessment scale (POSAS) and the Wake Forest University rating scale. RESULTS: The total patient score was 8.59 (range, 6-15) in the two-step incision group and 9.62 (range, 7-18) in the Koman incision group. A significant difference was found between the groups in the total PS score (P-value=0.034) but not in the total observer score. Our analysis found no significant difference in preoperative and postoperative Wake Forest University rating scale scores between the two-step and Koman incision groups. The time required for recovery prior to returning to work after surgery was shorter in the two-step incision group, with a mean of 29.48 days in the two-step incision group and 34.15 days in the Koman incision group (P=0.03). CONCLUSIONS: Compared to the Koman incision, the new two-step incision technique provides better aesthetic results, similar symptom improvement, and a reduction in the recovery time required before returning to work. Furthermore, this incision allows the surgeon to access a wide surgical field and a sufficient exposure of anatomical structures.
Cicatrix ; Hand* ; Humans ; Male ; Raynaud Disease ; Sympathectomy* ; Transplants ; Trees

Raynaud's phenomenon (RP) is characterized by reversible digital vasospasm triggered by exposure to cold or emotional stress. It manifests with unique 'triphasic' (pallor, cyanosis, erythema) or 'biphasic' (white, blue) color change. Clinically, RP is classified as primary RP, which does not have an underlying associated cause with a relatively benign course, and secondary RP, which predates various cause such as systemic connective tissue disease (CTD). Therefore, RP must be differentiated for detection of emerging CTD such as systemic sclerosis (SSc), systemic lupus erythematosus, and mixed conective tissue disease, ect. Nailfold capillaroscopy (NFC) is a non-invasive morphological study used routinely with antinuclear antibodies for parallel of secondary RP. A recent study showed NFC to be the best predictor of transition from a primary RP to secondary RP. The well-established role of NFC for early diagnosis of SSc, and potential for monitoring disease progression and predictive clinical complication makes NFC an important tool in research and clinical practice. In this paper, I present a recent update with emphasis on its possible role as a reliable diagnostic tool and biomarker in secondary RP.
Antibodies, Antinuclear ; Connective Tissue Diseases ; Cyanosis ; Disease Progression ; Early Diagnosis ; Humans ; Lupus Erythematosus, Systemic ; Microscopic Angioscopy* ; Raynaud Disease ; Scleroderma, Systemic ; Stress, Psychological

The aim of this study was to determine whether skin temperature measurement by digital thermography on hands and feet is useful for diagnosis of Raynaud's phenomenon (RP). Fifty-seven patients with RP (primary RP, n = 33; secondary RP, n = 24) and 146 healthy volunteers were recruited. After acclimation to room temperature for 30 min, thermal imaging of palmar aspect of hands and dorsal aspect of feet were taken. Temperature differences between palm (center) and the coolest finger and temperature differences between foot dorsum (center) and first toe significantly differed between patients and controls. The area under curve analysis showed that temperature difference of the coolest finger (cutoff value: 2.2degrees C) differentiated RP patients from controls (sensitivity/specificity: 67/60%, respectively). Temperature differences of first toe (cutoff value: 3.11degrees C) also discriminated RP patients (sensitivity/specificity: about 73/66%, respectively). A combination of thermographic assessment of the coolest finger and first toe was highly effective in men (sensitivity/specificity : about 88/60%, respectively) while thermographic assessment of first toe was solely sufficient for women (sensitivity/specificity: about 74/68%, respectively). Thermographic assessment of the coolest finger and first toe is useful for diagnosing RP. In women, thermography of first toe is highly recommended.
Adult ; Diagnosis, Differential ; Female ; Fingers/*physiology ; Humans ; Male ; Middle Aged ; ROC Curve ; Raynaud Disease/*diagnosis ; Sensitivity and Specificity ; Skin Temperature ; *Thermography ; Toes/*physiology