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Biomedical subjects

M B Stevens

Publications and source records attributed to M B Stevens.

At least 73 records · Page 4Linked to original sources

Acute abdominal complications of systemic lupus erythematosus and polyarteritis nodosa.

Fifteen (11 percent) of 140 patients with systemic lupus erythematosus (SLE) and five (31 percent) of 16 patients with polyarteritis nodosa (PA) developed disease-related signs and symptoms of an acute surgical abdomen. Peripheral vasculitis (p less than 0.025), nervous system involvement (p less than 0.05), ischemic necrosis of bone (p less than 0.05), thrombocytopenia (p less than 0.01), and circulating rheumatoid factor (p less than 0.01) are all significantly more prevalent in patients with SLE and abdominal involvement compared with those without an abdominal syndrome. Similarly, except for ischemic necrosis of bone, these same features are more prevalent in patients with PA and acute abdomens. Eleven (73 percent) of 15 patients with SLE had exploratory laparotomy for acute abdominal events. Nine were found to have evidence of intra-abdominal arteritis and only two had polyserositis without gross evidence of vasculitis. Eight (53 percent) of the 15 patients with SLE and acute abdomens died as a result of their abdominal crises. Six of the seven surviving patients represent those most recently seen and aggressively treated. All five patients with PA and acute abdomens required operative intervention and all had gross evidence of mesenteric arteritis with large ischemic segments resulting in infarction and perforation. All died in contrast to only two (18 percent) of the 11 PA patients without abdominal involvement.

Abdomen, Acute↗

Neurologic complications of primary Sjögren's syndrome.

Although peripheral nervous system disease has been well documented in Sjögren's syndrome (SS), central nervous system (CNS) involvement is considered distinctly uncommon. Sixteen patients with primary SS and CNS disorders not attributable to other causes were the subjects of this study. Cerebral manifestations, both focal and diffuse, as well as spinal cord disease, were observed. Peripheral vasculitis occurred in 12 patients (75%), 83% of whom had anti-Ro(SSA) antibodies. The high proportion of patients with concomitant peripheral vasculitis, and the observed association with antibodies to the Ro(SSA) antigen system which, in other studies, has been linked to vasculitis in SS, suggest that an immune vasculopathy may play a role in the pathogenesis of the CNS disease of SS.

Adult↗

The effect of cold exposure on diffusing capacity in patients with Raynaud's phenomenon.

Pulmonary hypertension is common in progressive systemic sclerosis (PSS), and the presence of "pulmonary Raynaud's phenomenon" has been postulated from a variety of evidence. In this study, the effect of cold-induced Raynaud's phenomenon on carbon monoxide diffusing capacity (Dco), an indicator of pulmonary capillary blood volume, was determined in patients with Raynaud's phenomenon with an without PSS. Cold exposure caused an increase in Dco in patients without PSS, but no change in patients with PSS. We concluded that patients with systemic sclerosis have an altered pulmonary vascular response to cold exposure compared with other Raynaud's patients, which may be due to either structural or functional abnormalities of the pulmonary vascular bed.

Adolescent↗

Survivorship in systemic lupus erythematosus: effect of antibody to extractable nuclear antigen.

The course of 81 patients with systemic lupus erythematosus (SLE) who had sera tested for antibody to extractable nuclear antigen (ENA) was studied to determine the effect of the presence of antiENA antibody on survivorship. There were no differences in percent survival between the patients with and without antibody to ENA or those with and without antibody to the ribonucleoprotein (RNP) component of ENA. We conclude that there is no prognostic advantage to the presence of either antiENA or antiRNP antibody in patients with SLE.

Adolescent↗

Rheumatoid arthritis in a Chippewa Band. I. Pilot screening study of disease prevalence.

The Mille Lacs Band of Chippewa Indians in central Minnesota was screened for rheumatoid arthritis, with a 77% completion rate of the reservation census. Rheumatoid arthritis was found in marked excess, namely 6.8% of those evaluated or, minimally, 5.3% of the total band if all persons had been evaluated with no additional cases identified. This relatively closed population thus provides an opportunity to assess genetic and environmental factors of significance in this disease.

Adolescent↗

HLA-DR characterization of a Chippewa Indian subpopulation with high prevalence of rheumatoid arthritis.

The adult population (N = 227) of a Chippewa Indian reservation in central Minnesota is characterized by a high prevalence of arthropathy with 7.1% having rheumatoid arthritis (RA). In a prospective study 168 reservation residents (74%) were evaluated. Complete HLA typing identified 57 haplotypes, many of which probably arose via HLA-A/B or B/D recombination. The number of founder haplotypes appeared to be about 20. The population frequency of DR4 (including DRw9, formerly designated 4 X 7) was 67%; for RA it was 100% (p less than 0.05). Apparent DR4 homozygotes, a number of the RA patients, and family members were selected for further study in a mixed lymphocyte culture (MLC) test and with 8th International Workshop cellular and serologic reagents. In MLC with homozygous typing cells (HTC), non of the DR4+ cells typed for any known HLA-D specificity, although they reacted to all DR4 antisera on the local panel. However, 8th International Workshop DR antisera revealed patterns of reactivity with non-DRf4 reagents consistent with the MLC. A minimum of three DR4 variants, one DRw9 variant, and a specificity related to both DR4 and DR2 are required to explain both the cellular and serologic reactions. For the present, we are designating the antigens as DR4.1chip, DR4.2chip, DR4.3chip, DR9chip, and DR(2 X 4)chip. No single variant of DR4 was characteristic of the RA patients in this Amer-indian population.

Adolescent↗

Sjögren syndrome: central nervous system manifestations.

We studied eight patients who had primary Sjögren syndrome and central nervous system (CNS) disorders that were not attributable to other causes. Focal cerebral deficits were observed in five patients. Aseptic meningoencephalitis was seen in five patients, recurrent in one. Spinal cord manifestations in three patients took several forms: acute transverse myelitis, chronic progressive myelopathy, and spinal subarachnoid hemorrhage. Precipitating antibodies to the Ro(SSA) cytoplasmic antigen were detected in the sera of seven of eight patients. This may be relevant to the pathogenesis of CNS disease in Sjögren syndrome, because there is a strong correlation between vasculitis and the presence of anti-Ro(SSA) antibodies in this connective tissue disorder.

Adult↗

The relationship between D-penicillamine--induced proteinuria and prior gold nephropathy.

Twenty-five patients with rheumatoid arthritis treated with D-penicillamine were retrospectively reviewed for signs of drug intolerance. Nine patients (36%) developed adverse drug reactions, the most common of which was proteinuria in six patients (24%). Comparative analysis of patients with and without penicillamine-induced proteinuria revealed the only significant correlate to be a previous history of gold nephropathy. Five (83%) of six patients who developed penicillamine-induced proteinuria had had gold-induced proteinuria; in contrast, only three (20%) of 19 who tolerated penicillamine had prior gold-induced proteinuria (p less than .01). HLA typing performed in five of the six patients with penicillamine nephropathy revealed DRw4 at a prevalence less than that expected for a population with rheumatoid arthritis, with DRw3 and/or B8 present in four patients. Further studies are in progress to determine whether a genetic predisposition is present. These data suggest that cautious observation is warranted in rheumatoid patients receiving D-penicillamine who have a prior history of gold nephropathy.

Adult↗

Ischemic bone necrosis in systemic lupus erythematosus. II. The early diagnosis of ischemic necrosis of bone.

It would appear from our experience thus far, that increased bone marrow pressure and altered venous drainage, as documented by venography, are present in all stages of ischemic necrosis of bone, including the pre-radiologic, and may be the earliest detectable changes in this disorder. This implies that any postulated pathogenetic mechanism must take into account the early appearance of significant venous derangement, although not necessarily indicating that the venous abnormalities are primary. The data indicate that the venous circulation participates in and undoubtedly contributes to the progression of the disorder. In addition the successful results of core decompression reported in Part II, lend support to such a pathogenetic sequence, and may well be due to the interruption of this vicious circle.

Adult↗

Hematological abnormalities in scleroderma. A study of 180 cases.

Hematological abnormalities in scleroderma indicate a specific complication of the disease itself, or an associated illness. Among 180 patients with scleroderma, anemia was detected in 25% and was attributed to chronic inflammatory disease (usually an overlap syndrome), bleeding mucosal telangiectases as part of the CREST syndrome, intestinal malabsorption, and microangiopathic hemolysis. Leukocytosis, present in 14%, was correlated with active myopathy and/or advanced visceral involvement while leukopenia was suggestive of a crossover with systemic lupus erythematosus. Thrombocytopenia was often a manifestation of microangiopathy, and thrombocytosis was associated with an arteritis or a tumor syndrome.

Anemia↗

The 'CREST' syndrome. Comparison with systemic sclerosis (scleroderma).

The CREST syndrome refers to a disorder comprising the manifestations of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. Thirteen CREST patients (two with CRST) were compared with 26 patients with systemic sclerosis but without the full manifestations of the CRST syndrome. No significant difference was found between the groups in the age of onset of Raynaud's phenomenon, degree of multiphasic digital color changes, ulcerations of fingers, sclerodactyly, or in the frequency of abnormal esophageal peristalsis or dysphagia. Laboratory results were similar, including the frequency of an elevated ESR. However, the CREST patients had a significantly lower frequency of arthralgia (54%) and arthritis (15%) than did those with scleroderma (88% and 65%, respectively). All but one of the CREST patients were women, which was a greater proportion than found among scleroderma cases (69%), and all were white (P less than .05). Most patients with the CREST syndrome had rather severe acrosclerosis. At last evaluation, four patients were chronically ill and three had died. The CREST and CRST syndromes are closely related disorders that seem to be part of the spectrum of systemic sclerosis.

Adult↗

Skeletal manifestations of polymyalgia rheumatica.

In a review of 39 patients with polymyalgia rheumatica (PMR), sternoclavicular and acromioclavicular arthropathy was noted in 15% and 10% of the patients, respectively. Diffuse clavicular swelling preceded the onset of PMR in one patient. Carpal tunnel syndrome was seen in four patients (10%).

Acromioclavicular Joint↗

Rheumatoid arthritis--a sequel to HBsAg hepatitis.

Reported here is the first case of classic rheumatoid arthritis emerging in the setting of hepatitis B surface antigen (HBsAG)-positive viral hepatitis. Polyfocal arthritis and myalgia, lymphadenopathy and constitutional symptoms were the presenting manifestations of anicteric hepatitis in this 23 year old man. Smooth muscles antibodies, HBsAg and "rheumatoid" factor were demonstrated initially. The hepatocellular disease, biopsy-proved, resolved completely and without recurrence; clinically and serologically. Latex test positivity persisted, increasing in titer, and polyarthritis progressed to fulfull criteria for classic rheumatoid arthritis, with erosions on roentgenogram and characteristic synovial disease. After 60 months of follow-up, the rheumatoid synovitis has continued to progress despite appropriate therapy. The arthritis of viral hepatitis and the significance of rheumatoid factor in association with hepatocellular disease are discussed.

Adult↗

The myocarditis of systemic lupus erythematosus: association with myositis.

Five patients with clinically overt myocarditis in the setting of systemic lupus erythematosus were analyzed in terms of associated clinical and serologic features. Myositis and antibodies to nuclear ribonucleoprotein (RNP) were present in all. A retrospective review in 140 consecutive patients with systemic lupus erythematosus, including three of these five, showed a highly significant association of myocarditis with myositis (P less than 0.0005). The presence of antibodies to RNP in this small group did not attain statistical significance (P less than or equal to 0.10). The pathologic findings in the one patient who died showed similar patterns of inflammation in both cardiac and skeletal muscle, suggesting the possibility of a generalized inflammatory process directed against striated muscle. Furthermore, although anti-RNP antibodies were found uniformly in these patients, their significance remains to be defined.

Adolescent↗