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Biomedical subjects

L H Calabrese

Publications and source records attributed to L H Calabrese.

At least 55 records · Page 3Linked to original sources

Limitations of invasive modalities in the diagnosis of primary angiitis of the central nervous system.

OBJECTIVE: Rheumatologists are often consulted to evaluate patients suspected of having primary angiitis of the central nervous system (PACNS). The diagnostic process relies heavily on interpreting the results of cerebral angiography and brain biopsy. We have assessed the operating characteristics of those invasive modalities in the diagnosis of PACNS: METHODS: The records of 30 consecutive patients referred for the evaluation of possible PACNS were retrospectively analyzed. Patients were evaluated on clinical grounds, and the diagnostic process was extended accordingly until a reasonable probability of a definable disease was present. All patients had cerebral angiography and/or brain biopsy. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were assessed for each of the following diagnostic tests: cerebral angiography, brain biopsy, cerebrospinal fluid (CSF) examination, and magnetic resonance imaging (MRI). RESULTS: The final diagnostic outcomes were: PACNS in 7, lymphoproliferative disease in 4, infection in 4, demyelinating disease in 2, reversible vasospastic disorder in 6, and a variety of other nonvasculitic conditions in 7. Cerebral angiography had less than 30% specificity and PPV for PACNS: Brain biopsy had limited sensitivity and NPV (53% and 70%, respectively). CSF examination and MRI, although sensitive, lacked specificity. CONCLUSION: In patients suspected of having PACNS, the results of invasive diagnostic modalities should be interpreted with caution. Accurate diagnosis should rarely rely on any single study and should only follow careful clinical, radiographic and pathologic correlation.

Brain↗

Vasculitis of the central nervous system.

Angiitis of the CNS remains a poorly understood form of vascular inflammatory disease. Primary angiitis of the CNS or PACNS, a disease once considered extremely rare, recently has been reported more frequently. Traditional views of PACNS held it to be a relentless and uniformly fatal disease if untreated, but it is now viewed as more heterogeneous, with some relatively benign subsets. Its pathophysiology is poorly understood. Secondary vasculitis of the CNS can occur with a variety of other conditions and diseases; each requires a different diagnostic and therapeutic approach. Regardless of whether primary or secondary CNS vasculitis is considered, an understanding of the limitations of currently available neurodiagnostic investigations is essential.

Central Nervous System↗

Chronic fatigue syndrome and a disorder resembling Sjögren's syndrome: preliminary report.

Chronic fatigue syndrome (CFS), as currently described in the working criteria proposed by the Centers for Disease Control and Prevention (Atlanta), may be associated with multiple, distinct, and possibly unique clinical and/or etiopathogenic subsets. Sjögren's syndrome (SS) is a disease of unknown etiology that is characterized by dryness of the mucous membranes and a variety of autoimmune phenomena and conditions. Subjective manifestations of SS such as neurocognitive dysfunction and fatigue have been stressed by some observers. We have detected a large number of patients with unrecognized SS-like illness in a clinical specializing in CFS and believe the relationship to be more than casual. From January 1991 through April 1992, 172 patients were evaluated for CFS; the SS cohort consisted of 27 females (mean age, 41.9 years). Sixteen of these patients had previously been found to have CFS by a physician, and 11 were self-referred. All patients complained of severe, dominating, chronic fatigue. Complaints of myalgia were prominent; 20 of 27 patients met the criteria for fibromyalgia. Neurocognitive complaints and/or a history of neuropsychiatric disease was frequent. Results of Schirmer's test were abnormal for 16 of 27, and results of minor salivary-gland biopsy were abnormal for 20 of 25. Antibodies to nuclear antigen were present in 16 of 27, but anti-Ro was present in only 1 of 21. In the SS group, 13 of 27 patients met eight or more CDC minor criteria for CFS, and 18 of 27 met six or more of the criteria.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Glycoforms of alpha 1-acid glycoprotein in sera of human immunodeficiency virus-infected persons.

In acute infections thus far studied, there is a relative increase in plasma protein glycoforms rich in biantennary complex type N-glycans (type I), while in some diseases with chronic inflammatory changes, there is increase in glycoforms with more branched N-glycans (type II). In sera of 109 human immunodeficiency virus (HIV)-infected persons, 38 rheumatoid arthritis patients, and 44 healthy subjects, the composition of alpha 1-acid glycoprotein (AGP) glycoforms was studied using crossed immunoaffinity electrophoresis with concanavalin A as a ligand. In patients in CDC classifications I, II, and III, distribution of AGP glycoforms was analogous to that in normal subjects. Type I alterations were observed in patients in group IV who had no signs of arthritis. Type II changes, analogous to those found in rheumatoid arthritis, were seen in group IV patients who developed arthritis. Most significant type I changes were associated with Pneumocystis carinii pneumonia (specificity, 100%; sensitivity, 96%).

Arthritis↗

Inclusion body myositis.

IBM remains a poorly understood form of idiopathic inflammatory myopathy, although great progress in the areas of clinical recognition and pathophysiology have been made recently. The question of whether therapy can favorably influence short- and/or long-term outcome is still unanswered. Several recent reports suggest some possibility of at least slowing progression with immunosuppressive therapy. Long-term therapeutic trials with goals that include stabilization, rather than improvements in strength, are urgently needed.

Humans↗

Effects of administration of an anti-CD5 plus immunoconjugate in rheumatoid arthritis. Results of two phase II studies. The CD5 Plus Rheumatoid Arthritis Investigators Group.

OBJECTIVE: To evaluate the safety and activity of an immunoconjugate of ricin A chain and anti-CD5 monoclonal antibody (anti-CD5 IC), with and without concomitant methotrexate and/or azathioprine, in the treatment of rheumatoid arthritis (RA). METHODS: Seventy-nine patients with active RA were enrolled in 2 prospective open-label protocols. RESULTS: Using composite criteria, response rates were 50-68% at 1 month and 22-25% at 6 months. Transient depletion of CD3/CD5 T cells was observed on days 2 and 5 of treatment, with reconstitution on day 15 or day 29. Treatment-associated adverse effects were common but resolved rapidly without sequelae. CONCLUSION: These findings suggest activity of anti-CD5 IC in active RA and warrant confirmation in a multicenter randomized study (currently underway).

Adult↗

Vascular nodules and plaques resembling chronic mucocutaneous candidiasis in a patient with a low interleukin 2 level.

A 21-year-old woman had a lifelong history of widespread friable vascular nodules and plaques. Her condition had previously been diagnosed as chronic mucocutaneous candidiasis because of her clinical appearance. Routine studies of immune function were performed in addition to a comparison of cytokine secretion in mononuclear leukocytes of our patient and normal controls. Interleukin 2 secretion by phytohemagglutinin-stimulated mononuclear leukocytes was deficient when compared with six healthy donors (< 300 pg/ml vs 2108 +/- 336 pg/ml). Interleukin 2 deficiency may be a causative factor in cases of unusual vascular proliferation. Treatment with methotrexate, which increases interleukin 2 activity and inhibits neovascularization in vitro, was helpful in our patient.

Adult↗

Early, aggressive therapy for rheumatoid arthritis: concerns, descriptions, and estimate of outcome.

The past few years have witnessed changing perceptions about rheumatoid arthritis (RA); it is now considered a serious systemic disease that confers not only physical and social morbidity but also earlier mortality. The long-term outcome of sequential monotherapy based on the therapeutic pyramid has been disappointing. A review of prognostic factors, acute disease activity measures, functional measures, and the results of preliminary trials with combination therapy suggests that specific goals of treatment can be established and that logical, aggressive treatment in early disease can be accomplished. These goals should include prompt control and continuous reduction of the active joint count to < or = 4 and normalization of acute-phase reactants. The "graduated-step paradigm" of treatment designed with these goals in mind is described, and a retrospective series that gives an estimate of outcome with its use is reported.

Adult↗

Benign angiopathy: a distinct subset of angiographically defined primary angiitis of the central nervous system.

OBJECTIVE: Primary angiitis of the central nervous system (PACNS) has been considered a rare and highly fatal disorder when untreated. In recent years the disease has been increasingly diagnosed by cerebral angiography, often without histologic confirmation. We have questioned whether cases of PACNS diagnosed on the basis of angiography alone are equivalent to histologically confirmed cases. METHODS: All cases of PACNS reported from 2 sources, including all cases reported in the English medical literature through January of 1990 as well as from the case records of the Cleveland Clinic Foundation were reviewed. Sixty-five variables were analyzed including demographic data, clinical signs and symptoms, treatment, outcome, diagnostic modalities and associated conditions. The cases were divided into 3 groups based on method of diagnosis. Group A referred to those diagnosed by angiography alone, but unconfirmed by histology, Group A & P for those who had positive histologic evidence angiitis confined to the central nervous system and a positive angiogram and Group P containing those diagnosed on the basis of histology who either never underwent angiography or who had angiographic findings other than arteritis. RESULTS: Cases of PACNS defined by angiography alone (Group A) appear often to be clinically distinctive, occurring more frequently in young women, often presenting with the sudden onset of focal neurologic deficit and associated with a relatively benign cerebral spinal fluid analysis. Patients defined in such manner frequently have a self-limited clinical course and may not require combination therapy with corticosteroids and cytotoxic drugs. CONCLUSION: We believe that PACNS is more clinically heterogeneous than is recognized and that a distinct and possibly more benign subset of disease that can be diagnosed on clinical and angiographic grounds has been identified.

Adult↗

Human immunodeficiency virus (HIV) infection and arthritis.

A variety of inflammatory arthritic conditions are observed in the setting of HIV infection. The epidemiology of these disorders is a point of current controversy, although it appears that several unique syndromes are clinically associated. The pathogenesis of these disorders remains unclear, but we hope that further work in this area will lend important insights into the mechanisms of both HIV-associated and non-HIV associated rheumatic disease. The overall management of such patients is based on recognizing the underlying HIV infection and the judicious use of antirheumatic drug therapy. Rheumatologists need to be aware of the natural history of HIV infection and its clinical manifestations.

Arthritis, Infectious↗

Maintenance of CD4+ cells by thymopentin in asymptomatic HIV-infected subjects: results of a double-blind, placebo-controlled study.

OBJECTIVE: To assess the efficacy and safety of thymopentin in HIV-infected patients who had not yet developed AIDS. DESIGN: Patients were stratified into asymptomatic or symptomatic groups and randomized to receive either thymopentin (50 mg) or placebo, subcutaneously, double-blind for 24 or 52 weeks, three times a week. SETTING: Patients were enrolled at three sites (two hospital clinics and one private practice). PATIENTS: Of 91 HIV-seropositive patients (52 asymptomatic and 39 symptomatic) from whom HIV could be isolated from peripheral blood, 45 were enrolled for 24 weeks and 46 for 52 weeks of double-blind evaluation. MAIN OUTCOME MEASURES: Virological, immunological and clinical evaluations were performed before and during treatment. RESULTS: Thymopentin-treated asymptomatic patients had more CD4+ cells, as demonstrated by a greater area under the percentage CD4+ cells curve (P = 0.03) and a shorter median time to a 20% increase in percentage of CD4+ cells (P = 0.04) in the first 24 weeks, with similar trends in the 52-week study. By 24 weeks no asymptomatic thymopentin-treated and two placebo-treated patients (9.1%, Kaplan-Meier estimate) had progressed to constitutional symptoms (P = 0.12; two-tailed Wilcoxon-Gehan test), with only one further progression in a placebo-treated patient in the subset followed for 52 weeks. Symptomatic patients receiving thymopentin or placebo were similar in both CD4+ cell levels and disease progression (two progressions to AIDS in each group). No serious adverse effects attributable to thymopentin were observed. CONCLUSIONS: These results, if confirmed, indicate that thymopentin, by maintaining CD4+ cells, could slow or arrest immune decline and consequent disease progression at the asymptomatic stage of HIV infection.

Amino Acid Sequence↗

Inclusion body myositis: analysis of 32 cases.

Inclusion body myositis is characterized by an insidious onset, progressive indolent course, and is generally felt to be refractory to standard therapy for myositis. We reviewed the charts of 32 patients with muscle biopsy findings suggestive of inclusion body myositis. The average time from symptom onset to diagnosis was 37 months, but initially 40% were incorrectly diagnosed. Twenty-eight patients (88%) were classified as definite or probable inclusion body myositis and were treated with various combinations of prednisone and immunosuppressive agents. Sixty-eight percent of those treated experienced a decrement in function and muscle strength. Three patients exhibited longterm improvement while 12 patients experienced delayed progression, defined by short term improvement in strength or a stable functional class, All of these patients received therapy, 5 in the form of methotrexate and prednisone. All untreated patients deteriorated clinically. In summary, (1) inclusion body myositis is a clinically distinct entity which is frequently misdiagnosed initially. (2) While clinical improvement with therapy is rare, our observations support recent reports that therapy may be associated with a slower rate of clinical progression. (3) Optimal therapy remains uncertain, but the use of low dose methotrexate and prednisone may warrant further study.

Adult↗

The family physician's contribution to the care of patients in the final stages of HIV infection.

This article concludes the monthly series developed from the AOA Task Force on AIDS Writers' Workshop, held August 16 to 18, 1991, in New York. The workshop was sponsored by an education grant from Burroughs Wellcome. This series has provided brief clinical updates and perspectives on the human immunodeficiency virus (HIV). Readers may request tear sheets from the AOA editorial offices.

Acquired Immunodeficiency Syndrome↗