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At least 19 recordsLinked to original sources
PubMed1978

Free serum ribonucleoprotein in mixed connective tissue disease and other connective tissue diseases.

Free ribonucleoprotein (RNP) was found by means of a hemagglutination inhibition assay in 21 sera from 11 of 25 patients with mixed connective tissue disease (MCTD) who had 299 sera studied. Free RNP tended to appear when anti-RNP antibody titers fell or when prednisone was initiated or increased. Five of 72 SLE patients (211 sera) had free RNP in at least one serum. Three of them had anti-RNP antibodies in other sera. Free serum RNP was found in one of 20 patients with scleroderma and in two of seven patients with connective tissue disease "overlap" syndromes without anti-RNP.

Antibodies, Antinuclear↗
PubMedDate not supplied

Gastrointestinal systemic sclerosis in serologic mixed connective tissue disease.

Mixed connective tissue disease is a clinical entity defined by overlapping features of progressive systemic sclerosis, systemic lupus erythematosus, polymyositis, rheumatoid arthritis, and distinct serologic findings. Esophageal dilatation and dysmotility have been the only gastrointestinal manifestations reported. Three patients with serologic findings of mixed connective tissue disease and extensive gastrointestinal involvement compatible with the changes found in progressive systemic sclerosis are presented. Gastrointestinal manifestations of progressive systemic sclerosis are reviewed and were found to be indistinguishable from the findings in these patients.

Adult↗
PubMedDate not supplied

Pulmonary hypertension in a child with mixed connective tissue disease.

Mixed connective tissue disease (MCTD) is characterized by high titers of antibody to ribonucleoprotein (RNP) in patients with features of several rheumatic diseases. We describe a child whose MCTD included arthritis, Raynaud's phenomenon, mucocutaneous ulcerations, sclerodermatous skin changes, restrictive lung disease, reduced carbon monoxide pulmonary diffusing capacity, abnormal esophogeal motility, and severe pulmonary hypertension. High antibody titers to ds-DNA and RNP were present. Clinical improvement followed therapy with prednisone and cyclophosphamide. Improvement in the degree of pulmonary hypertension was documented by repeat cardiac catheterization 8 months after the initiation of combination therapy.

Adolescent↗
PubMed1978

The hand in mixed connective tissue disease.

The mixed connective tissue disease syndrome has been described in the medical literature. The clinical and serological characteristics of the syndrome are defined in this paper. The hands of these patients differ from the hands of patients with systemic lupus, rheumatoid arthritis, or systemic sclerosis. In 10 patients there were no erosive changes on radiological examination and all 10 patients had Raynaud's phenomenon. The most striking finding was tightness in the flexors. Mild cases of flexor tightness improved with systemic steroids. One patient with severe flexor tightness required surgical release of adhesions from a chronic inflammatory process of fascia, muscle, and tenosynovium. Biochemical studies showed an abnormal collagen pattern that may be distinct for mixed connective tissue disease.

Adolescent↗
PubMedDate not supplied

Mixed connective tissue disease in siblings.

Mixed connective tissue disease (MCTD) was diagnosed in a brother and sister, and 18 additional family members spanning three generations were studied to detect evidence of autoimmune disease. Symptoms or signs of MCTD without complete expression of the disease were found in 8 relatives of the original cases. Antibodies to ribonucleoprotein and high-titer antinuclear antibodies were found only in the affected siblings. Tests for rheumatoid factor were positive in 9 of 17 relatives of the patients; the titers ranged from 1:160 to 1:2560. The brother and sister with MCTD had an identical HLA genotype--11,12/2,12. The same genotype was inherited by 3 of their siblings, who had impressive rheumatic complaints. This report emphasizes the association between inflammatory connective tissue disease and a specific HLA type within a single kindred.

Adult↗
PubMed1976

Mixed connective tissue disease.

Three patients with mixed connective tissue disease (MCTD) had clinical features that included a high incidence of Raynaud phenomenon, arthritis, myositis, and swollen hands. The diagnostic laboratory test result was the presence of high titers of antibody to extractable nuclear antigen. These antibody titers are notably reduced or abolished in patients with MCTD when the tanned red blood cells that are used in the test are pretreated with ribonuclease. Speckled antinuclear antibodies were present in all patients. Patients with MCTD have a low incidence of renal disease, are responsive to treatment with prednisone, and have a good prognosis.

Adolescent↗
PubMed1978

Isolated trigeminal sensory neuropathy: early manifestation of mixed connective tissue disease.

A young woman with mixed connective tissue disease (MCTD) had an isolated trigeminal sensory neuropathy as an early manifestation of the disease. Raynaud phenomenon occurred almost synchronously with the onset of trigeminal neuropathy and was followed by myositis, diffuse hand swelling, synovitis, and increased ribonucleoprotein antibody. Mixed connective tissue disease has overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis, and is differentiated from them by high-titer antibody to ribonucleoprotein.

Adult↗
PubMed1978

The arthritis of mixed connective tissue disease.

Twenty patients with mixed connective tissue disease were followed for 5 years. Arthritis occurred in all 20 patients, being the presenting complaint in 11 patients. The joints most frequently involved were the proximal interphalangeal (PIP), metacarpophalangeal (MCP), wrists, metatarsophalangeal (MTP), and knee; the distribution tended to be symmetrical, mimicking early rheumatoid arthritis. Joint deformities occurred in 6 patients, but apart from 1 patient with arthritis mutilans, significant functional impairment was not encountered. Radiologically small punched out bone erosions, asymmetrically distributed, were the most characteristic finding; other notable changes were aseptic necrosis, tuft erosions, and periarticular calcification. Joint effusions were non-inflammatory, the cellular content was predominantly lymphocytic and the C3 level was normal. Most cases were controlled with non-steroidal anti-inflammatory agents and invariably responded to prednisone less than or equal to 7.5 mg/day.

Adolescent↗
PubMed1977

Immune-complex glomerulonephritis in a patient with mixed connective tissue disease.

Renal involvement and hypocomplementemia in mixed connective tissue disease are reported to be rare. A patient is described here with mixed connective tissue disease and persistently low serum C'3 levels in whom renal insufficiency and nephrotic syndrome developed secondary to immune-complex glomerulonephritis. Light microscopy of the renal biopsy specimen showed predominantly a membranous lesion. Immunofluorescent staining showed granular deposition along the basement membrane of immunoglobulin G, immunoglobulin M, fibrinogen and C3. Electron microscopy showed numerous electron-dense deposits along the glomerular capillary membrane and in the mesangium.

Adult↗
PubMed1977

[Sharp's syndrome (mixed connective tissue disease). Clinical aspects diagnosis and prognosis].

Sharp syndrome (mixed connective tissue disease) is a distinct rheumatic syndrome with symptoms of various connective tissue diseases (rheumatoid arthritis, systemic lupus erythematodes, progressive systemic sclerosis, polymyositis and others). 15 patients with mixed connective tissue disease are described. The clinical picture and diagnostic criteria are evaluated and the course of the disease, treatment and prognosis are discussed.

Adolescent↗
PubMed1975

[Sharp's syndrome: a new entity amongst the connective tissue diseases?].

Sharp and coworkers have coined the name "Mixed connective tissue disease" to an association of symptoms seen in different connective tissue diseases. Those symptoms are associated with a special antibody directed against ribonucleoproteins (RNA-P). The presence of this antibody accounts for a high titer of antinuclear fluorescent antibody, of the speckled type. The most frequents symptoms in the association described by Sharp are Raynaud's phenomenon, swollen fingers, non deforming arthritis. We have observed those symptoms without any further symptoms of connective tissue disease, but with the presence of anti-RNA-P antibody. We suggest to coin the name of Sharp's syndrome to that clinical association. The significance of anti-RNA-P, and their correlation with a remarkable benignity of the disease, is discussed.

Antibodies, Antinuclear↗
PubMed1976

Symptomatic Sjögren's syndrome in mixed connective tissue disease.

Twelve of 25 patients with mixed connective tissue disease complained of xerostomia and/or ocular symptoms of keratoconjunctivitis sicca. In addition to the clinical features of mixed connective tissue disease, all 12 patients had high titers of antibody to the ribonuclease-sensitive component of the extractable nuclear antigen. Eight patients had both clinical xerostomia and keratoconjunctivitis sicca, one had keratoconjunctivitis sicca and salivary gland enlargement, while there had xerostomia but no ocular complaints. Sjörgren's syndrome was confirmed in all 12 patients by means of Schirmer's tests, Rose Bengal staining tests, salivary gland scintiscans, radionuclide excretion studies in saliva, parotid sialographies, and lip biopsies. At least three of these tests were abnormal in all patients.

Collagen Diseases↗
PubMed1977

Immunopathology of skeletal muscle. The value of direct immunofluorescence in the diagnosis of connective tissue disease.

Whitaker and Engel in 1972 first described immunoglobulin deposition in muscle biopsy specimens in connective tissue disorders. In order to confirm and extend their observations and in the hope of identifying features that may differentiate skeletal muscle involvement in connective tissue diseases from other neuromuscular diseases, a series of 80 muscle biopsy specimens were examined using direct immunofluorescence. Three distinctive direct immunofluorescence patterns consisting of vascular, sarcolemma-basement membrane, and fiber staining were identified in skeletal muscle biopsy specimens from 35 patients, 31 of whom had a connective tissue disease. A vascular staining pattern showing a granular deposition of immunoglobulin or complement was seen in 15 patients, all of whom had a connective tissue disease. Routine stains generally did not reveal vessel abnormalities. A sarcolemma-basement membrane staining pattern was demonstrated around the sarcoplasmic membrane in 29 cases. Twenty-six of these patients had a connective tissue disease. There was no correlation with inflammation, fiber necrosis, or degree of connective tissue replacement. Fibers staining for immunoglobulin or complement, seen in 14 cases, generally occurred in morphologically normal fibers. Thirteen of these patients had a connective tissue disease. Since the pathologic change in muscle in the collagen vascular diseases often consists of the nonspecific findings of focal fiber necrosis frequently without inflammatory infiltrates, direct immunofluorescence may be useful in the diagnosis and classification of muscle diseases in the collagen vascular disorders. Furthermore, the findings of immunoglobulin deposition either within vessels or within individual muscle fibers suggest that immunological mechanisms may be responsible for muscular abnormalities in the connective tissue diseases.

Basement Membrane↗
PubMed1978

Neuropsychiatric problems in mixed connective tissue disease.

A group of 20 patients with mixed connective tissue disease, followed for up to five years, was found to have a 55 per cent incidence of neuropsychiatric problems. An aseptic meningitis-like syndrome was the most common presentation and was rapidly responsive to corticosteroid therapy. Other findings were psychosis, convulsions, peripheral neuropathy, trigeminal neuropathy and cerebellar ataxia. An abnormal cerebrospinal fluid was found in five patients; mild pleocytosis, an increased protein content and a first phase colloidal gold curve were the main abnormalities. These neuropsychiatric problems have not been a cause of mortality in this group of patients with mixed connective tissue disease.

Adrenal Cortex Hormones↗
PubMed1976

Pneumatosis intestinalis in association with connective tissue disease.

Pneumatosis intestinalis in association with connective tissue diseases is an unusual combination whose pathogenesis is not yet understood. Furthermore, steroid medication, often used to treat these diseases, may itself cause pneumatosis. Three cases of scleroderma, systemic lupus erythematosus, and amyloidosis in association with pneumatosis and without prior steroid therapy are presented. The small vessel occlusive pathologic processes in these diseases may cause focal areas of mucosal ischemia resulting in small, perhaps transient ulcerations that allow gas to enter the gut wall from the lumen.

Adult↗
PubMed1978

Mixed connective tissue disease in childhood. Relationship Sjögren's syndrome.

Mixed connective tissue disease (MCTD) seems to be a distinct entity that has some manifestations of systemic lupus erythematosus, scleroderma, polymyositis, and Sjögren's syndrome and is serologically characterized by the presence of an antibody to ribonucleoprotein. We report the cases of three children with MCTD with high titers of antibody to ribonucleoprotein. Two fulfilled criteria of lupus erythematosus, two had polymyosis; all three had suggestive features of scleroderma, fulfilled criteria for the diagnosis of juvenile rheumatoid arthritis, and had Sjögren's syndrome. Additional superimposed features of another connective tissue disease should arouse suspicion of MCTD. All three of our patients responded adequately to corticosteroid treatment that makes recognition of this entity by the pediatrician all the more important.

Adolescent↗
PubMed1978

Mixed connective tissue disease in children.

A diagnosis of Mixed Connective Tissue Disease (MCTD) had been made in five juveniles in the past two years. All have antibodies to the ribonucleoprotein (RNP) component of extractable fluorescence pattern. No child has had life-threatening complications, though three have required steroids to control their symptoms. Antibodies to ENA were looked for in the other sub-groups of Juvenile Chronic Arthritis (JCA); only two of 75 patients with chronic iridocyclitis and antinuclear antibodies had weakly positive tests.

Adolescent↗