- Digital Ischemia as an Unusual Manifestation of Hodgkin's Lymphoma. [Journal Article]
- CRCase Rep Hematol 2018; 2018:1980749
- Digital ischemia is associated with atherosclerotic, thromboembolic, or connective tissue diseases. Less often, it can be related to malignancy. Paraneoplastic vascular acrosyndromes (Raynaud's syndr...
Digital ischemia is associated with atherosclerotic, thromboembolic, or connective tissue diseases. Less often, it can be related to malignancy. Paraneoplastic vascular acrosyndromes (Raynaud's syndrome, acrocianosis, and acronecrosis) are associated with adenocarcinoma and less frequently with hematological malignancies. We report the case of a 45-year-old male, smoker, with a 10-day history of pain, cyanosis, and progressive digital necrosis in both hands. In the previous four months, he noticed painless mass in the right axillary gap, drenching night sweats, and weight loss. Physical examination at admission highlighted necrotic lesions on the distal phalanges of both hands (except the thumbs), enlarged lymph nodes in right axillary, and right supraclavicular gaps. Arteriography of upper limbs demonstrated a distal stop in all bilateral digital arteries. Digital ischemia was interpreted as a paraneoplastic phenomenon after other common etiologies were ruled out. Amputation of three phalanges was required due to necrosis. Biopsy of axillary nodes demonstrated nodular sclerosis classical Hodgkin's lymphoma (HL). The patient started conventional ABVD protocol (doxorubicin, bleomycin, vinblastine, and dacarbazine). After 6 cycles, he remained asymptomatic and symptoms of digital ischemia were completely resolved. It was concluded that the presence of acral vascular syndromes should alert the physician about the possibility of underlying malignant disease. Prompt investigation and treatment should be rapidly performed to avoid digital sequelae.
- High burden of skin sclerosis is associated with severe organ involvement in patients with systemic sclerosis and systemic sclerosis overlap syndrome. [Journal Article]
- RIRheumatol Int 2018 Sep 11
- The objective of this study is to investigate the impact of skin sclerosis burden on an internal organ involvement over a 1-year period, as measured by time-adjusted accrual-modified Rodnan skin scor...
The objective of this study is to investigate the impact of skin sclerosis burden on an internal organ involvement over a 1-year period, as measured by time-adjusted accrual-modified Rodnan skin score (TA-mRSS), and to evaluate association between TA-mRSS patterns and laboratory tests in patients with systemic sclerosis (SSc). This prospective study was conducted at Siriraj Hospital (Bangkok, Thailand) during the November 2013-November 2016. SSc patients by ACR/EULAR 2013 or ACR 1980 criteria were eligible. TA-mRSS was classified as low, intermediate, or high, and then compared between groups. Correlation between the arithmetic mean of laboratory tests and TA-mRSS was assessed by multiple linear regression analysis. A total of 118 patients, with 81.4% women, median (IQR) age 49.8 (43.8, 55.1) years, disease duration from onset of non-Raynaud symptoms to first visit of 3.3 (1, 6.8) years, 78% dcSSc, and 75.3% anti-Scl-70 positivity, were analyzed. TA-mRSS over 1 year ranged from 0 to 37.44. The high skin sclerosis burden group had a median TA-mRSS > 7.26 (> 67th percentile). Patients with high TA-mRSS were dcSSc, high initial and average mRSS, and had tendon friction rub, digital ischemic complications, usual interstitial pneumonia, diastolic dysfunction, gastrointestinal dysmotility, and low serum albumin. In multiple linear regression analysis, the arithmetic mean of hemoglobin (B = - 1.007, 95% CI - 1.779 to - 0.236), erythrocyte sedimentation rate (B = - 0.078, 95% CI - 0.126 to - 0.029), serum glutamic oxaloacetic transaminase (B = 0.073, 95% CI 0.026-0.12), creatine phosphokinase (B = 0.012, 95% CI 0.003-0.021), and albumin (B = - 4.117, 95% CI - 6.958 to - 1.276) were associated with TA-mRSS. This study found a higher cumulative course of mRSS over a 1-year period to be significantly associated with severe internal organ involvement.
- [Digital necrosis revealing cold agglutinin disease: Treatment with rituximab]. [Journal Article]
- ADAnn Dermatol Venereol 2018 Sep 06
- CONCLUSIONS: Cold agglutinin disease is a rare type of auto-immune hemolytic anemia. Following exposure to cold, paroxysmal cutaneous signs are frequent. The disease may be either primary or secondary with B-cell lymphoproliferative disorder, auto-immune disease or infection. A thorough workup is required. To date, the treatment combining the best positive response rate and good safety is rituximab in weekly perfusions over a 1-month period.
- A Phenotype of Primary Open-angle Glaucoma with Systemic Vasospasm. [Journal Article]
- JGJ Glaucoma 2018 Sep 04
- CONCLUSIONS: We describe a POAG subtype with APON and systemic vascular instability, predominantly female in their sixth decade of life who demonstrate progressive glaucomatous visual field damage at low intraocular pressure. We suggest that this clinical picture represents an important phenotype of POAG, and that identification and further study of it will help guide diagnosis and development of individualized treatments.
- Evolving symptoms of Raynaud's phenomenon in systemic sclerosis are associated with physician and patient-reported assessments of disease severity. [Journal Article]
- ACArthritis Care Res (Hoboken) 2018 Aug 21
- CONCLUSIONS: Patients identify with distinct patterns of SSc-RP that may relate to progression of the obliterative microangiopathy of SSc. Difficulty distinguishing discrete SSc-RP attacks from persistent digital ischaemia in advanced SSc could influence diary-based approaches to assessing SSc-RP with implications for clinical trials. This article is protected by copyright. All rights reserved.
- Successful Endovascular Treatment for Aortic Thrombosis Due to Primary Antiphospholipid Syndrome: A Case Report and Literature Review. [Journal Article]
- VEVasc Endovascular Surg 2018 Aug 09; :1538574418791355
- A 60-year-old man with a history of Raynaud's phenomenon presented with bilateral intermittent claudication and an ulcer on his right toe. The ankle-brachial index of the right and left legs was 0.77...
A 60-year-old man with a history of Raynaud's phenomenon presented with bilateral intermittent claudication and an ulcer on his right toe. The ankle-brachial index of the right and left legs was 0.77 and 0.75, respectively. Laboratory data showed prolongation of the activated partial thromboplastin time and a positive result on the lupus anticoagulant test. Computed tomography angiography revealed isolated infrarenal aortic stenosis with irregular surface and noncalcified plaques. Intravascular ultrasonography examination demonstrated a noncalcified, irregular, and mobile plaque, suggestive of abdominal aortic thrombosis. In addition to anticoagulant and dual antiplatelet therapy, endovascular treatment was performed. A total of three 40-mm-long balloon-expandable stents were successfully implanted on a 15-mm balloon. The final angiography showed good results except for minimal plaque shifting in the terminal aorta. Three months later, the ulcer resolved and a final diagnosis of primary antiphospholipid syndrome (APS) was made. Clinicians should recognize that APS can affect the abdominal aorta, leading to aortic thrombosis. Endovascular treatment may be the one good treatment option for this rare condition.
- [Cold and White - Hypothenar Hammer Syndrome]. [Journal Article]
- PPraxis (Bern 1994) 2018; 107(16):912-916
- Cold and White - Hypothenar Hammer Syndrome Abstract. Hypothenar hammer syndrome (HHS) is a condition caused by digital ischemia as a result of repeated trauma to the little finger. Routine diagnosis...
Cold and White - Hypothenar Hammer Syndrome Abstract. Hypothenar hammer syndrome (HHS) is a condition caused by digital ischemia as a result of repeated trauma to the little finger. Routine diagnosis should include a detailed medical history and a physical examination including Allen's test. Imaging of vascular lesions can be done initially by acral plethysmography and duplex sonography, or directly in the context of angiography (gold standard). Early diagnosis enables effective therapeutic strategies and preventing permanent sequelae. The optimal treatment options are selected depending on the intensity of symptoms, ranging from conservative methods, secondary prevention, through a local thrombolysis up to operational measures.
- What does it mean if a patient is positive for anti-Jo-1 in routine hospital practice? A retrospective nested case-control study. [Journal Article]
- FF1000Res 2018; 7:698
- Background: It is widely believed that patients bearing auto-antibodies to histidyl tRNA synthetase (anti-Jo-1) very likely have a connective tissue disease including myositis and interstitial lung...
Background: It is widely believed that patients bearing auto-antibodies to histidyl tRNA synthetase (anti-Jo-1) very likely have a connective tissue disease including myositis and interstitial lung disease. The value of positive tests in low disease prevalence settings such as those tested in routine care is unknown. We sought to determine the value of anti-Jo-1 auto-antibodies in routine practice. Methods: Our study was a nested case control study within a retrospective cohort of all patients tested for anti-ENA our hospital, from any hospital department, between January 2013 and December 2014. Data was extracted from electronic records of anti-Jo-1 positive patients and randomly selected ENA negative patients (ratio of 1:2), allowing for a minimum follow up of at least 12 months after first testing. Results: 4009 samples (3581 patients) were tested. Anti-ENA was positive in 616 (17.2%) patients, 40 (1.1%) were anti-Jo-1 positive. Repeat ENA testing was done for 350/3581 (9.8%) patients (428 of 4009 (10.7%) samples) and in 7/40 (17.5%) of anti-Jo-1 positive patients. The median interval between the first and second request was 124 days (inter-quartile range 233 days). The frequencies of interstitial lung disease (ILD), myositis and Raynaud's were comparable for anti-Jo-1 positive patients (n=40) and 80 randomly selected ENA negative controls. Positive tests led to additional diagnostic testing in the absence of clinical disease. Sensitivity of Jo-1 for ILD was 50% (CI 19-81%), specificity 68% (CI 59-77%), positive predictive value 12.5% (CI 4 to 27%) and negative predictive value 93.8% (CI 86-98%). Of 10 (25%) patients with high anti-Jo1 levels, 3 had ILD, one myositis and two a malignancy (disseminated melanoma and CML). Conclusion: Anti-Jo-1 is uncommon in a heterogenous hospital population and is only weakly predictive for ILD. Repeated test requests were common and potentially unnecessary indicating that controls over repeat requests could yield significant cost savings.
- Novel case of anti-synthetase syndrome. [Journal Article]
- EJEur J Rheumatol 2018 Jun 20; :1-3
- Anti-synthetase syndrome (AS) is a heterogeneous group of systemic autoimmune diseases associated with anti-aminoacyl-transfer RNA synthetases. These inflammatory myopathies present with a constellat...
Anti-synthetase syndrome (AS) is a heterogeneous group of systemic autoimmune diseases associated with anti-aminoacyl-transfer RNA synthetases. These inflammatory myopathies present with a constellation of symptoms including myositis, arthritis, Raynaud's phenomenon, and interstitial lung disease (ILD). We present a novel case of a 44-year-old female, who presented with Anti-OJ AS with severe myopathy and rhabdomyolysis without evidence of ILD, which, in our literature review and to the best of our knowledge, has not been previously reported. Furthermore, our patient was initially misdiagnosed, highlighting the paucity of cases and physicians' unfamiliarity with this disease. After her diagnosis was confirmed, the patient was successfully treated with high-dose steroids and transitioned to azathioprine, and she continues to do well. This case report emphasizes a novel presentation of the rarely diagnosed AS. We also discuss the significant overlap between the inflammatory myopathies and consolidate relevant pathophysiology and current trends in the management of this disease.
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- The march of pluripotent stem cells in cardiovascular regenerative medicine. [Review]
- SCStem Cell Res Ther 2018 Jul 27; 9(1):201
- Cardiovascular disease (CVD) continues to be the leading cause of global morbidity and mortality. Heart failure remains a major contributor to this mortality. Despite major therapeutic advances over ...
Cardiovascular disease (CVD) continues to be the leading cause of global morbidity and mortality. Heart failure remains a major contributor to this mortality. Despite major therapeutic advances over the past decades, a better understanding of molecular and cellular mechanisms of CVD as well as improved therapeutic strategies for the management or treatment of heart failure are increasingly needed. Loss of myocardium is a major driver of heart failure. An attractive approach that appears to provide promising results in reducing cardiac degeneration is stem cell therapy (SCT). In this review, we describe different types of stem cells, including embryonic and adult stem cells, and we provide a detailed discussion of the properties of induced pluripotent stem cells (iPSCs). We also present and critically discuss the key methods used for converting somatic cells to pluripotent cells and iPSCs to cardiomyocytes (CMs), along with their advantages and limitations. Integrating and non-integrating reprogramming methods as well as characterization of iPSCs and iPSC-derived CMs are discussed. Furthermore, we critically present various methods of differentiating iPSCs to CMs. The value of iPSC-CMs in regenerative medicine as well as myocardial disease modeling and cardiac regeneration are emphasized.