Venous gangrene of the upper extremity.
Kaufman B R,Zoldos J,Bentz M,Nyström N A
Annals of plastic surgery
Venous gangrene of the upper extremity is a rare entity and is the result of massive occlusion of all venous outflow of the extremity. The syndrome is strongly associated with hypercoagulable states including malignancy, low cardiac output states, and hereditary or acquired hematological abnormalities. Diagnosis can be straightforward but must be made early in the course of the process for treatment to be effective. Treatment has historically produced only modest results, and patients continue to suffer a high morbidity and mortality. We present a series of 6 patients with venous gangrene or impending venous gangrene of the upper extremities--a relatively large series. Two patients suffered from malignancy, 3 patients suffered from low-flow cardiac states, and 1 patient suffered from an overdose of calcium channel blockers. Hematological abnormalities included heparin-induced thrombocytopenia and thrombosis in 3 patients, activated protein C resistance in 1 patient, and lupus anticoagulant in 1 patient. Three patients experienced other major venous thrombotic complications, two of whom died (renal and cerebral venous infarction). Venous gangrene of the upper extremity remains a rare occurrence but one in which early identification and intervention may lead to improved outcomes.
Antibodies and vascular involvement in inflammatory joint disease: clinical relevance.
Vittecoq O,Jouen-Beades F,Tron F,Le Loët X
Joint bone spine
The vascular endothelium is a common target of inflammatory joint disease. Autoimmune diseases including rheumatoid arthritis, systemic lupus erythematosus, and Sjögren's syndrome can be responsible for a spectrum of vascular disorders that encompasses vasculitis, thrombosis and/or atheroma associated with the antiphospholipid syndrome, and vascular damage caused by cryoglobulin deposition. These mechanisms can coexist, particularly in lupus patients. Joint disease is sometimes the presenting manifestation in primary vasculitis. Autoantibodies are detectable in most patients with vascular involvement and inflammatory joint disease. They are not merely markers for vascular involvement: in vitro and in vivo data suggest that some autoantibodies may contribute to the genesis of endothelial lesions, together with other factors. For instance, evidence of pathogenic effects has been found for antineutrophil cytoplasmic antibody (ANCA), most notably with antimyeloperoxidase or antiproteinase-3 specificity, in small-vessel vasculitides (Wegener's granulomatosis, Churg-Strauss syndrome, and microscopic polyangiitis); for immune complexes, particularly those containing cryoglobulins, in vasculitides secondary to CTDs; and for circulating anticoagulant and anticardiolipin antibodies, above all anti-beta2-glycoprotein I, in antiphospholipid syndrome. Antibodies to annexin V, modified lipoproteins, and endothelial cells may be of interest; their clinical relevance is unclear, however, and no standardized assays are available, so thatthese antibodies are not looked for in everyday practice. When deciding which antibody tests should be performed in a given patient, the circumstances surrounding the onset of the vasculopathy should be borne in mind. In patients with previous CTD, the tests are selected based on the diagnosis. In contrast, in a patient with no previous diagnosis, a vasculopathy can be either primary or secondary to undiagnosed CTD or to antiphospholipid syndrome: consequently, a broader array of tests is needed in this situation.
10.1016/s1297-319x(01)00309-8
Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases.
Matsuyama Tomomi,Kuwana Masataka,Matsumoto Masanori,Isonishi Ayami,Inokuma Shigeko,Fujimura Yoshihiro
Thrombosis and haemostasis
To clarify the pathogenic processes of thrombotic microangiopathies (TMAs) in patients with connective tissue disease (CTD), we analysed clinical characteristics and plasma ADAMTS13 levels in 127 patients with CTD-TMAs, including patients with systemic lupus erythematosus (SLE), systemic sclerosis, polymyositis/dermatomyositis, and rheumatoid arthritis (RA), and 64 patients with acquired idiopathic thrombotic thrombocytopenic purpura (ai-TTP). Plasma levels of ADAMTS13 activity, antigen, and inhibitors were determined by enzyme immunoassays. IgG type anti-ADAMTS13 antibodies were also detected by immunoblots using purified ADAMTS13. ADAMTS13 activity was significantly decreased in CTD-TMAs, regardless of the underlying disease, but the frequency of severe deficiency (defined as <0.5% of normal) was lower in CTD-TMA patients than in ai-TTP patients (16.5% vs. 70.3%, p < 0.01). Severe deficiency of ADAMTS13 activity was predominantly detected in patients with RA- and SLE-TMAs, and was closely associated with the presence of anti-ADAMTS13 IgG antibodies. CTD-TMA patients with severe deficiency of ADAMTS13 activity appeared to have lower platelet counts and better therapeutic outcomes. At least two phenotypic TMAs occur in patients with CTDs: a minor population with deficient ADAMTS13 activity caused by neutralising autoantibodies, and a major population with normal or moderately reduced activity. Classifying CTD-TMAs by ADAMTS13 activity may be useful in predicting the clinical course and therapeutic outcomes, as patients with moderately reduced activity are likely to have more prominent renal impairment and poor prognoses.
10.1160/TH08-12-0825
The clinical significance of a shortened activated partial thromboplastin time in patients with connective tissue disease.
Habe Koji,Wada Hideo,Mizutani Kento,Matsushima Yoshiaki,Kondo Makoto,Yamanaka Keiichi
Clinical rheumatology
INTRODUCTION:Connective tissue disease (CTD) patients have been reported to have an increased risk of venous thromboembolism (VTE). Deep venous thrombosis represents a potential emergency that may have a fatal outcome. The D-dimer test is the most widely accepted screening marker for VTE; however, elevation of the plasma D-dimer level without demonstrable thrombosis sometimes accompanies CTD activity itself, infection, and other conditions. Thus, the accuracy of a diagnosis of VTE based on a D-dimer test result is lower in CTD patients. The activated partial thromboplastin time (APTT) test is a very common and simple test. METHOD:The medical records of 535 CTD patients were retrospectively investigated. The following data were extracted: APTT, D-dimer, thrombotic events, laboratory data, and systemic corticosteroid therapy. RESULTS:The rates of thrombotic events and VTE were significantly increased in patients with a shortened APTT (< 26 s) (PSAPTT) in comparison to those without a shortened APTT (p = 0.004, 0.0009, respectively). The number of PSAPTTs was significantly increased in patients with VTE in comparison to those without VTE (p = 0.0009). In the diagnosis of VTE in CTD patients, the specificity and positive predictive value (PPV) of the D-dimer test were 71.6% and 83.8% and 12.7% and 19.4%, respectively. The combination of a shortened APTT and elevated plasma D-dimer level improved the specificity and PPV to 94.7% and 97.3% and to 25.0% and 36.4%, respectively. CONCLUSIONS:For the evaluation of possibility of accompanying VTE in CTD patients, APTT shortened was useful and should be evaluated with careful attention. KEY POINTS:• Regarding the specificity for diagnosing VTE in CTD patients, a shortened APTT showed a value (84.3%) comparable or superior to that of the D-dimer test. • The combination of a shortened APTT and elevated D-dimer level improved the specificity of the diagnosis of VTE in CTD patients to (94.7% or 97.3%) in comparison to the D-dimer test alone (71.6% or 83.8%). • The positive predictive value of the combination of a shortened APTT and plasma D-dimer elevation for the diagnosis of VTE in CTD patients increased to 25.0% or 36.4%. • In the management of CTD patients, physicians should pay attention when they encounter patients with a shortened APTT, as it may indicate VTE.
10.1007/s10067-021-05781-w
Immunity and early atherosclerosis in the course of systemic lupus erythematosus, mixed connective tissue disease and antiphospholipid syndrome.
Haładyj Ewa,Paradowska-Gorycka Agnieszka,Felis-Giemza Anna,Olesińska Marzena
Reumatologia
Atherosclerosis is a chronic inflammatory disease of the arteries associated with various risk factors that promote lipid abnormalities, development and progression of atherosclerotic lesions, plaque rupture, and vascular thrombosis. Atherosclerosis is accelerated in autoimmune diseases. Non-invasive investigations showed increased intima-media thickness (IMT), carotid plaque, and coronary artery calcifications in patients with antiphospholipid syndrome, systemic lupus erythematosus and mixed connective tissue disease compared to controls. The balance between the proinflammatory and anti-inflammatory cytokines allows the immune equilibrium to be maintained. In autoimmune diseases the prevalence of proinflammatory factors leads to premature atherosclerosis. This review presents complementary knowledge on innate and adaptive immunity, cytokines and the role of inflammasomes in progression of early atherosclerosis.
10.5114/reum.2016.62473
Clinical Manifestations and Mechanisms of Autoimmune Disease-Related Multiple Cerebral Infarcts.
Sun Li-Li,Tang Wen-Xiong,Tian Min,Zhang Lu,Liu Zun-Jing
Cell transplantation
It is important to investigate the clinical characteristics and identify the stroke mechanisms of patients with autoimmune disease-related stroke, which are necessary for early etiology diagnosis, accurate treatment and preventive strategies. In this article we retrospectively studied eight cases of acute ischemic stroke associated with autoimmune diseases, and without competing conventional stroke etiologies. The characteristics of stroke (clinical and radiological features), the laboratory tests especially serum D-dimer levels (as a marker of hypercoagulable state), and embolic signals on transcranial Doppler were evaluated for all eight patients. High-resolution magnetic resonance imaging (HRMRI), which can help to evaluate vasculitis was performed in four patients. The possible underlying mechanisms of these cases were discussed based on these manifestations. As a result, autoimmune diseases in our study included systemic lupus erythematosus (=5), mixed connective tissue disease (=1), central nervous system vasculitis (=1), and Takayasu arteritis (=1). All eight patients presented with acute infarction lesions in ≥2 vascular territories. Most patients presented with numerous small and medium infarction lesions located in the cortical and subcortical areas. Multiple stroke mechanisms were involved in these cases, including hypercoagulability (=4), cardiac embolism (=1) and vasculitis (=3). Embolic signals could be detected on transcranial Doppler in all three stroke mechanisms. In conclusion, our study revealed the characteristics of autoimmune disease-related stroke. For patients with multiple acute cerebral infarcts within non-single arterial territories, autoimmune disease is an important etiology not to be neglected. Multiple stroke mechanisms were involved in these cases.
10.1177/0963689719846838