Biomedical subjects
M B Stevens
Publications and source records attributed to M B Stevens.
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.
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%).
Malignant disease mimicking arthritis of the hip.
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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.
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.
The pseudothrombophlebitis syndrome.
Sixty-two patients with popliteal or calf synovial cysts defined by arthrography are presented, of whom 34 had the pseudothrombophlebitis (PTP) syndrome. While the clinical manifestations of PTP may closely mimic thrombophlebitis, including the presence of calf pain, swelling and warmth, and a positive Homans' sign, the helpful descriminating features include the presence of inflammatory joint disease (91%), concomitant pain and swelling of the knee (94%), a demonstrable knee effusion (91%) and the absence of deep venous tenderness or cord. Except for seven patients with large intact Baker's cysts, the PTP syndrome was associated with synovial cyst dissection (18 patients), rupture (5 patients), or both (4 patients). Of those with arthrographically demonstrated Baker's cysts, only 27 (44%) had a palpable mass in the popliteal fossa and another 4 (12%) a mass in the calf. Popliteal fossa pain was noted in 22 (35%). Thus, clinical features are far less sensitive than arthrography in the diagnoses of both synovial cysts and pseudothrombophlebitis. Among patients with rheumatoid arthritis there were no differences in the severity or duration of disease or the extra-articular features in patients with and without synovial custs or the PTP syndrome. The therapeutic implications of pseudothrombophlebitis are of major significance with respect to the avoidance of anti-coagulation and the prompt response to intra-articular corticosteroids. Possible mechanisms of synovial cyst formation and unusual presentations of synovial cysts are discussed, and the literature is reviewed.
Intervertebral disc space infection: another low back syndrome of the young.
Pyogenic disc space infection is a rare and unappreciated cause of low back pain. Reported herein is a young man with chronic low back pain and the HLA-B27 antigen which initially led to the diagnosis of ankylosing spondylitis. However, serial lumbar radiographs and bone scan established an inflammatory lesion involving the L2-L3 intervertebral disc space, and persistently normal sacroiliac joints. A needle biopsy and culture of the disc space yielded Staphylococuss aureus. Treatment with antibiotics, bed rest and back bracing resulted in a complete resolution of symptoms and healing of the vertebral lesion. It is the purpose of this report to review the clinical, laboratory and radiographic features of intervertebral disc space infection as well as pitfalls in its diagnosis.
Letter: Granulomatous synovitis and Crohn's disease.
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Ischemic necrosis of bone in systemic lupus erythematosus.
Twenty-three patients with systemic lupus erythematosus (SLE) and ischemic bone necrosis are reported. All patients had received corticosteroids prior to the onset of ischemic necrosis, although one patient had received none for 13 years previously. Nineteen (83%) patients had multiple bone lesions including the femoral heads in 21 (91%) which were bilaterally involved in 15. In addition, humeral heads were affected in seven patients and the tibial plateaus, in three. The most striking feature of this group was the high incidence of Raynaud's phenomemon present in 14 (61%) of the 23 patients. Furthermore, central nervous system involvement was present in 10 (43%) patients, more prominent in those without Raynaud's (67%) than in those with vasospasm (29%). Thus, 20 of the 23 patients, or 87%, evidenced vascular abnormalities either in the form of Raynaud's phenomenon and/or systemic vasculitis. The pathogenesis of ischemic bone necrosis is discussed. In SLE, vasospasm or vasculitis, or both, augmented by corticosteroid therapy, could impede the microcirculation and result in the ischemic lesion.
Neuropsychiatric manifestations of systemic lupus erythematosus: diagnosis, clinical spectrum, and relationship to other features of the disease.
1. Among patients with SLE, 71 (51%) had significant neuropsychiatric problems during the course of the disease. In 52 (37%), the nervous system manifestations were secondary to SLE. 2. The most frequent manifestations were psychiatric dysfunction, seizures, long tract signs, cranial neuropathy, and peripheral neuropathy. 3. Psychiatric abnormalities secondary to SLE were characterized by organic features (present in 22 of 24) and by the association of neurologic lesions which were often diffuse or multifocal. 4. An abnormal cerebrospinal fluid was found in 32% of neuropsychiatric episodes in which specimens were obtained. The most frequently abnormal study was the electroencephalogram (71%), and the least frequent was the brain scan (8%). These studies did not correlate with specific clinical patterns. 5. In 63% of the patients, NP manifestations preceded the diagnosis of SLE or occurred within the first year of diagnosed disease, and in most episodes were associated with evidence of clinical and/or serologic activity of the underlying illness. 6. Only two clinical features showed significant and striking correlations with neuropsychiatric involvement, namely vasculitis and thrombocytopenia. The possible pathogenic implications have been discussed. 7. Only 2 of the 140 patients were felt to have steroid-induced psychoses. In approximately one-half of the NP episodes secondary to SLE, patients were receiving no corticosteriods on presentation. Of those developing while patients were on steroids, the majority occurred on low doses or after tapering from higher levels. 8. The immediate prognosis for improvement in neuropsychiatric function was good with 84% of episodes showing complete or partial resolution. Corticosteroids appeared to be of benefit in a substantial number of patients although their precise role is difficult to quantitate. 9. Five and 10 years survivals for the overall population were 94% and 82%, respectively. There were no significant differences in survival for patients with or without nervous system involvement.
Urinary fibrin split products in lupus nephritis.
Antigen-antibody interaction and fibrin deposition at the glomerular basement membrane can release activators that ultimately convert fibrin into degradation products of varying molecular size. Thus, increased urinary fibrinogen/fibrin split products (FSP) might be expected in active lupus nephritis. A longitudinal study was done on 27 patients with biopsy-proven lupus nephritis. Those patients who were persistent FSP excretors were found to be at a significantly greater risk of progressive nephritis and renal death than those who were FSP negative. It is suggested that FSP excretion in lupus nephritis, particularly on a continued basis and despite normalization of other renal disease parameters, is a grave prognostic sign.
Colonic perforations in systemic lupus erythematosus.
Five patients with systemic lupus erythematosus (SLE), four of whom died with colonic perforations, are reported. Perforation of the colon constituted the most frequent cause of death among 107 patients with SLE admitted to the Rheumatic Disease Unit during a three year period. All five patients with colonic perforation had clinical and laboratory manifestations of active SLE in addition to the abdominal syndrome. Most striking was evidence of active arteritis in all patients with either central nervous system involvement and/or peripheral arteritis, in addition to that found in the gastrointestinal tract. Hyperglobulinemia and rheumatoid factor as well as antinuclear antibodies were present at some time in all patients. The abdominal syndrome was characterized by the insidious onset of lower quadrant pain which was intermittent and colicky. Although direct abdominal tenderness was eventually present in all patients, rebound tenderness and hypoactive bowel sounds were variable and abdominal rigidity occurred only in one patient and late in the course. The differential diagnosis of abdominal pain in SLE is reviewed and possible mechanisms for the production of colonic perforations are discussed. It is suggested that the presence of rheumatoid factors in conjunction with circulating immune complexes may be the pathogenetic mechanism via the production of a mesenteric arteritis.
Penicillin hypersensitivity in systemic lupus erythematosus.
The prevalence of positive immediate-type skin tests to penicillin antigens among patients with systemic lupus erythematosus (SLE) was compared with hospitalized controls without SLE and found not to differ significantly. On the other hand, we confirmed previous reports that the prevalence of histories of prior penicillin reaction was significantly higher in SLE patients than among controls. This is felt to be related to the primary disease process, and we conclude that patients with SLE do not have an increased risk of IgE-mediated allergic reactions to penicillin.
The arthropathy of Behçet's disease.
Behçet's disease, originally described as a triad of ocular inflammation and oral and genital ulcerations, is better understood as a mulitsystem disease involving skin, joints, central nervous system, large bowel, and peripheral veins. This report of a 49-year-old female patient with uveitis and recurrent orogenital lesions, polyarthritis, and skin lesions, serves as introduction to a review of articular involvement in Behçet's disease. Included is complete synovial fluid analysis with measurement of intraarticular complement. Arthropathy, occurring in at least one-half of reported patients, is usually polyarticular and asymmetrical. It affects knees and ankles most frequently and rarely produces loss of function or deformity. During exacerbations synovial fluid appears inflammatory with polymorphonuclear leukocytosis greater than 25,000 cells/mul; synovial fluid complement is consistently elevated. Histologically the synovium shows increased vascularity with perivascular lymphocyte infiltration. Etiology and treatment of Behçet's disease are briefly discussed.
Destructive monarthritis and granulomatous synovitis as the presenting manifestations of Crohn's disease.
A twenty-two-year-old woman and a fourteen-year-old boy with Crohn's disease presented with progressive monarticular arthritis with radiologic evidence of altered articular cartilage and subchondral bone. In one individual, granulomatous inflammation of the synovium was demonstrated. Intestinal symptoms were not prominent. In both individuals, the development of the monarthritis led to the discovery of the underlying inflammatory bowel disease.
Limited anti-DNA antibody specificity in systemic lupus erythematosus.
Twenty-one sera from 11 patients with systemic lupus erythematosus were titrated with 125I-ss-calf thymus DNA to determine their maximum capacity to bind DNA. Only five sera were capable of binding greater than 90% of the input DNA. The remaining sixteen sera showed maximum DNA binding levels between 35% and 85%. The inability of these sera to bind all of the input DNA is shown to be dependent on variations in the molecular weight of the DNA antigen. The possibility that these differences in DNA binding reflect differences in antigenic specificity is discussed.