Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A retrospective survey of all patients with positive anti-centromere antibody (ACA) determinations over a 5-year period was undertaken. Of the 52 patients identified, seven denied symptoms of Raynaud's phenomenon, all of whom had antibody titres of 2560 or less. One of these showed a 16-fold increase in titre with the onset of Raynaud's, 12 years after original presentation. The remaining 45 patients experienced Raynaud's on cold exposure. Within this group, seven developed thrombotic vascular disease, five of whom were associated with an ACA titre of 20,480 or above. Another two with similar high ACA titres developed hyper-reninaemic hypertension, suggesting disease of the renal vasculature and associated ischaemia. Where it was possible to ascertain, seven of eight with high titre ACA developed sclerodactyly. In contrast, this was not seen in those with low titre ACA of 2560 or less and who also denied Raynaud's phenomenon.
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PMID:The titre of anti-centromere antibodies: its relationship to Raynaud's phenomenon and vascular occlusion. 273 Sep 83

Sixteen patients with progressive systemic sclerosis (PSS), including 3 with the "CREST" (calcinosis, Raynaud's, esophageal dysfunction, sclerodactyly, and/or telangiectasias) variant, were evaluated with resting M-mode echocardiography and noninvasive measurements of cardiac output at rest and during submaximal exercise to determine the nature and extent of any cardiovascular impairment. No patient had arterial hypertension, significant renal impairment, clinical evidence of large vessel coronary artery disease, or severe pulmonary dysfunction. The duration of disease was 1 to 12 years (9 to 30 for patients with the CREST variant). Echocardiographic abnormalities included increased right ventricular dimension (3 patients), reduced left ventricular ejection fraction (3 patients), and pericardial effusion (3 patients). Cardiac index (CI) and stroke volume index (SVI) at rest were similar for patients and controls. Patients and controls were exercised to similar heart rates (130 +/- 3 vs 124 +/- 4; p, NS). Total peripheral resistance (TPR) was higher for patients (1123 +/- 81 vs 810 +/- 44 dyn X s X cm-5) and their mean SVI failed to increase significantly compared with sitting rest values (30 +/- 2 vs 35 +/- 3 ml/m2). The control subjects had the expected increase in SVI (36 +/- 2 vs 51 +/- 5; p less than 0.01). Ten patients with an abnormal hemodynamic response to exercise had a normal echocardiographic circumferential fiber shortening (VCF) or ejection fraction (EF) at rest. The data indicate that PSS patients have a greater degree of cardiovascular dysfunction than would be predicted from clinical data and laboratory evaluation of cardiovascular and pulmonary function at rest. Multiple mechanisms, including right and left ventricular dysfunction and abnormal vasoconstrictor activity, are likely to contribute to the reduction in exercise capacity seen in patients with PSS.
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PMID:Cardiovascular function in patients with progressive systemic sclerosis (scleroderma). 621 13

Over 100 cases of disorders closely resembling classic autoimmune diseases have been reported among patients who were injected or implanted with a diverse group of chemicals including paraffins, vegetable oils or silicone. Most cases have occurred in silicone breast implant recipients, especially those who received their prostheses 2-10 years prior to onset of symptoms. A high proportion of patients exhibit classic signs and symptoms of Sjogren's syndrome or scleroderma. Affected patients typically experience some combination of fatigue, myalgia, joint pain, sicca syndrome (dry eyes and mouth), synovitis, rash, alopecia, muscular weakness or lymphadenopathy, and autoantibody formation. Less commonly, patients may have the CREST syndrome (calcinosis, Raynaud's phenomena, esophageal hypomotility, sclerodactyly and telangiectasias), hypertension, pulmonary fibrosis, or central nervous system pathology.
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PMID:Silicone-reactive disorder: a new autoimmune disease caused by immunostimulation and superantigens. 828 1

A 56-year-old man was admitted to the nephrology unit with a short (6-week) history of severe hypertension that necessitated renal replacement therapy within 7 days after admission. Renal biopsy showed features of thrombotic microangiopathy in arterioles and small arteries with occluding thrombi. The skin was unremarkable at the time of admission. Progressive skin lesions with scleroderma, telangiectasia, sclerodactyly, and generalized cutaneous sclerosis developed within 4 weeks and the specific skin changes were found on skin biopsy. On admission antinuclear antibody titers were high (1:10, 240) with a nucleolar pattern, and PM-Scl antibodies (1:5, 120) were present. In the present case the diagnosis of scleroderma renal crisis was made in vivo by renal biopsy. Renal and skin biopsies documented that renal lesions may precede the clinically manifest skin lesions of progressive systemic sclerosis.
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PMID:Scleroderma renal crisis as a presenting feature in the absence of skin involvement. 832 94

Two girls with mixed connective tissue disease (MCTD) were treated in our hospital in the past 5 years. Patient 1, a 10-year-old girl presenting with migratory arthralgia, had an initial diagnosis of juvenile rheumatoid arthritis. Muscle weakness with elevated levels of creatine kinase and liver enzymes, sclerodactyly, Raynaud's phenomenon and heliotrope sign developed subsequently in the following 3 years. Patient 2, a 13-year-old girl, had been treated for suspected systemic lupus erythematosus since 9 years of age. She presented with lymphadenopathy, arthralgia, pericardial effusion, and paralytic ileus. The symptoms waxed and waned. Sclerodactyly, Raynaud's phenomenon, proteinuria, and hypertension were also noted. Both patients had high serum titers of antinuclear antibody (speckled pattern, 1:5120) and were seropositive for antiribonuclear protein antibody. Intravenous immunoglobulin, prednisolone, cyclosporine A, and nonsteroidal anti-inflammatory drugs (NSAIDs) were given to patient 1. Patient 2 received cyclosporine A, prednisolone, and methylprednisolone pulse therapy. The disease has been well controlled for 2 years by low-dose immunosuppressants and NSAIDs. MCTD is a rare juvenile rheumatic disease: early identification and appropriate treatment can improve the disease outcome.
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PMID:Childhood mixed connective tissue disease. 1077 31

Capillaroscopy is a method with substantial value for diagnosis and differentiation of primary and secondary Raynaud's phenomenon in rheumatic diseases. The most specific finding is in systemic sclerosis--the so-called "scleroderma pattern." which is characterized by the presence of dilated capillaries, hemorrhages, avascular areas, and neoangiogenesis. Similar changes are found in patients with dermatomyositis, overlap syndromes, and others and are termed "scleroderma-like pattern." For the development of these patterns, the most specific finding in the early phase is appearance of dilated capillaries. Capillaroscopic changes in connective autoimmune diseases are specific and differ significantly from those of that can be found in other diseases. Diseases of social importance such as diabetes mellitus and arterial hypertension often present as comorbidity in patients with rheumatic diseases. In diabetes mellitus, the capillaroscopic examination does not show dilated capillaries until the advanced stages of the disease. In the late stages of connective tissue disease, a loss of capillaries is typical. In addition, in diabetes mellitus, the diabetic stiff-hand syndrome and sclerodactyly are common complications, which have to be differentiated from similar signs in rheumatic diseases, and capillaroscopic examination appears to be useful in these situations. In arterial hypertension, a reduced capillary density in different body regions has been observed in patients with established disease as well as in preclinical stages. Analogous phenomenon of reduction in the nail-fold area has also been observed in a group of patients with essential hypertension, none of whom previously received hypertensive drugs.
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PMID:The specificity of capillaroscopic pattern in connective autoimmune diseases. A comparison with microvascular changes in diseases of social importance: arterial hypertension and diabetes mellitus. 1977 65

An association between connective tissue growth factor (CTGF) gene dimorphism at -945 (CTGF*-945C/G) and systemic sclerosis (SSc) has been reported with inconclusive results. We performed this study to determine whether such an association exists among Thai patients with SSc. DNA samples were taken from 50 Thai SSc patients (diffuse SSc in 39 and limited SSc in 11) and 99 healthy controls for determination of CTGF*-945C/G dimorphism by polymerase chain reaction (PCR) using specific oligonucleotide primers. The associations between the genotype frequencies, clinical manifestations and auto-antibodies were determined as well. When compared with the controls, SSc patients had no significantly higher frequencies of the GG genotype (44.0% vs 39.4%, p = 0.60), G allele (63.0% vs 65.2%, p = 0.80) or G phenotype (82.0% vs 90.9%, p = 1.0). There was no association between the presence of the GG genotype and clinical manifestations (pulmonary fibrosis, sclerodactyly, digital pitting scars, telangiectasia and pulmonary arterial hypertension), or the presence of auto-antibodies (anti-Scl-70, anti-SSA/Ro, and anti-RNP). In conclusion, we found no association between CTGF*-945C/G dimorphism and Thai SSc patients.
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PMID:Lack of CTGF*-945C/G Dimorphism in Thai Patients with Systemic Sclerosis. 2204 8

A 68-year-old female who had Raynaud phenomenon for a decade was admitted to our hospital in January 2012. She complained of sclerodactyly and scleroderma that did not extend past the elbows. She also had fingertip ulcers that repeatedly disappeared and recurred for several years. Blood tests showed that she was anti-centromere antibody positive. Therefore, she was diagnosed with limited cutaneous systemic sclerosis. Two months after diagnosis, she returned to our hospital because she experienced dyspnea on exertion and exacerbation of her fingertip ulcers. Chest X-rays revealed cardiac enlargement, an echocardiography showed tricuspid regurgitation with an increased tricuspid pressure gradient (91 mmHg) and right heart catheterization showed a mean pulmonary arterial pressure of 59 mmHg. Chest computed tomography and lung perfusion scintigraphy showed no abnormalities. She was then diagnosed with pulmonary arterial hypertension associated with systemic sclerosis. She improved rapidly with daily treatments of prednisolone in addition to warfarin, bosentan and beraprost sodium. This is a rare case of rapidly progressive pulmonary arterial hypertension associated with systemic sclerosis that can be markedly improved with early diagnosis and treatment.
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PMID:[Rapidly progressive pulmonary arterial hypertension associated with systemic sclerosis : a case report]. 2381 75

Red face is not a rare finding in patients with connective tissue disorders. The malar eruption is the most frequent cutaneous manifestation of systemic lupus erythematosus (LE). This condition is more apparent among fair-skinned individuals, and it usually appears after sun exposure. A very important clinical sign is that nasolabial folds remain free of any erythematous or other changes. With subacute cutaneous LE, sun exposure can provoke a red face that resembles the malar eruption of systemic LE. The typical clinical findings of chronic cutaneous LE are the discoid lesions. There is a clinical form of chronic cutaneous LE called erythema perstans faciei. This form is purely erythematous, and it usually appears on the face. Other rare "red face" forms of chronic cutaneous LE are LE tumidus and LE telangiectaticus. Red face is not typical of systemic sclerosis, but facial telangiectasias are frequent, especially with CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. The differential diagnoses of other red face manifestations are easy due to the additional findings. Telangiectasias are accompanied by calcinosis, sclerodactyly, digital ischemia, and Raynaud disease. Many studies mention telangiectasias as markers of the severity of the systemic sclerosis, the disease duration, any pulmonary arterial hypertension, and any esophageal involvement. Purple- or violet-colored upper eyelids are the hallmark and one of the first clinical signs that is helpful for the diagnosis of dermatomyositis. This violaceous to dusky erythema can extend over the whole face and the upper aspects of the trunk. Erythematous changes on the face that are different from those of the heliotrope sign which occurs with dermatomyositis may be observed in both sun-exposed skin and non-sun-exposed skin. Malar and facial erythema, linear extensor erythema, V-sign or shawl sign, and other photodistributed eruptions can also appear.
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PMID:The red face revisited: connective tissue disorders. 2431 89

Renal involvement occurs in 1%-12% in juvenile systemic sclerosis (JSSc) patients, mainly with arterial hypertension, proteinuria and scleroderma renal crisis. We report herein a patient who presented nephrotic syndrome (NS) as the first manifestation of JSSc with focal segmental glomerulosclerosis (FSGS). A female patient presented steroid-sensitive NS at the age of 12 years. At 14 years, she had orbital and lower limbs edema, arterial hypertension, sclerodactyly and proximal skin sclerosis. Moderate capillary dilation and mild focal devascularization were observed in nailfold capillaroscopy, compatible with early stage of scleroderma (scleroderma pattern). Percutaneous renal biopsy guided by ultrasound revealed focal segmental glomerulosclerosis and direct immunofluorescence were negative. Therefore, she fullfilled the provisional classification criteria for JSSc. Patient was treated with oral 25-hydroxyvitamin D (800 IU/day), methotrexate (0.5mg/kg/week) and amlodipin (0.15 mg/kg). Prednisone (60 mg/m2/day) was administered for 4 consecutive weeks, followed by alternate-day (40mg/m2) for 2 consecutive months, with tapering for 4 months and then stopping this medication. Currently she is being treated with methotrexate 15 mg/week, without edema and proteinuria. In conclusion, we reported a rare case of NS with FSGS as the first manifestation of scleroderma. Therefore, renal biopsy is mandatory in JSSc patients with sustained proteinuria or NS.
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PMID:[Nephrotic syndrome as the first manifestation of juvenile systemic scleroderma.] 2544 Jul 9


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