Charles H. Mckenna

Relapsing polychondritis. Survival and predictive role of early disease manifestations.

To define the natural history of relapsing polychondritis, the probability of survival and causes of death were determined in 112 patients seen at one institution. By using covariate analysis, early clinical manifestations were identified that predicted mortality. The 5- and 10-year probabilities of survival after diagnosis were 74% and 55%, respectively. The most frequent causes of death were infection, systemic vasculitis, and malignancy. Only 10% of the deaths could be attributed to airway involvement by chondritis. Anemia at diagnosis was a marker for decreased survival in the entire group. There was an interaction between other disease variables and age in determining their impact on outcome. For patients less than 51 years old, saddle-nose deformity and systemic vasculitis were the worst prognostic signs. For older patients, only anemia predicted outcome. The need for corticosteroid therapy did not influence survival.

Enhanced coronary vasa vasorum neovascularization in experimental hypercholesterolemia.

Coronary arteries contain a network of vasa vasorum in the adventitia. The three-dimensional anatomy of the vasa vasorum in early coronary atherosclerosis is unknown. This study was designed to visualize and quantitate the three-dimensional spatial pattern of vasa vasorum in normal and experimental hypercholesterolemic porcine coronary arteries, using a novel computed tomography technique. Animals were killed after being fed either a high cholesterol diet (n = 4) or a control diet (n = 4) for 12 wk. The proximal left anterior descending coronary artery was removed from the heart, scanned, and reconstructed, and quantitation of vasa vasorum density was performed. Two different types of vasa vasorum were defined: first-order vasa vasorum ran longitudinally parallel to the vessel and second-order originated from first-order vasa circumferentially around the vessel wall. Compared with controls in hypercholesterolemic coronary arteries, there was a significant increase in the area of the vessel wall (3.86+/-0.22 vs. 8.07+/-0.45 mm2, respectively, P < 0.01) and in the density of vasa vasorum (1. 84+/-0.05/mm2 vs. 4.73+/-0.24/mm2; respectively, P = 0.0001). This occurred especially by an increase of second-order vasa vasorum and disorientation of normal vasa vasorum spatial pattern. This study suggests that adventitial neovascularization of vasa vasorum occurs in experimental hypercholesterolemic coronary arteries and may be a part of the early atherosclerotic remodeling process.

Epidemiology of Systemic Lupus Erythematosus and Other Connective Tissue Diseases in Rochester, Minnesota, 1950 Through 1979

The incidence and prevalence rates of connective tissue disease syndromes in Rochester, Minnesota, from 1950 through 1979 are reported. The incidence of definite systemic lupus erythematosus (SLE) has not increased since 1960. The incidence of SLE in the elderly population was higher than that in previous reports. Rates of SLE and discoid lupus erythematosus were approximately equal. Other diagnoses (in decreasing order of frequency) were suspected lupus erythematosus, scleroderma, drug-induced lupus, and overlapping connective tissue disease syndromes. The 10-year survival of patients with definite SLE was decreased, and the survival of patients with suspected SLE was the same as that of the general population.

Adventitial vasa vasorum in balloon-injured coronary arteries: visualization and quantitation by a microscopic three-dimensional computed tomography technique.

OBJECTIVES The objective of this study was to examine the quantitative response of the adventitial vasa vasorum to balloon-induced coronary injury. BACKGROUND Recent attention has focused on the role of vasa vasorum in atherosclerotic and restenotic coronary artery disease. However, the three-dimensional anatomy of these complex vessels is largely unknown, especially after angioplasty injury. The purpose of this study was to visualize and quantitate three-dimensional spatial patterns of vasa vasorum in normal and balloon injured porcine coronary arteries. We also studied the spatial growth of vasa vasorum in regions of neointimal formation. A novel imaging technique, microscopic computed tomography, was used for these studies. METHODS Four pigs were killed 28 d after coronary balloon injury, and four pigs with uninjured coronary arteries served as normal controls. The coronary arteries were injected with a low-viscosity, radiopaque liquid polymer compound. Normal and injured coronary segments were scanned using a microscopic computed tomography technique. Three-dimensional reconstructed maximum intensity projection and voxel gradient shading images were displayed at different angles and voxel threshold values, using image analysis software. For quantitation, seven to 10 cross-sectional images (40 normal and 32 balloon injured cross-sections) were captured from each specimen at a voxel size of 21 microm. RESULTS Normal vasa vasorum originated from the coronary artery lumen, principally at large branch points. Two different types of vasa were found and classified as first-order or second-order according to location and direction. In balloon-injured coronary arteries, adventitial vasa vasorum density was increased (3.16+/-0.17/mm2 vs. 1.90+/-0.06/mm2, p = 0.0001; respectively), suggesting neovascularization by 28 d after vessel injury. Also, in these injured arteries, the vasa spatial distribution was disrupted compared with normal vessels, with proportionally more second-order vasa vasorum. The diameters of first-order and second-order vasa were smaller in normal compared with balloon-treated coronary arteries (p = 0.012 first-order; p < 0.001, second-order; respectively). The density of newly formed vasa vasorum was proportional to vessel stenosis (r = 0.81, p = 0.0001). Although the total number of vasa was increased after injury, the total vascular area comprised of vasa was significantly reduced in injured vessels compared with normals (3.83+/-0.20% to 5.42+/-0.56%, p = 0.0185). CONCLUSIONS Adventitial neovascularization occurs after balloon injury. The number of new vessels is proportional to vessel stenosis. These findings may hold substantial implications for the therapy of vascular disease and restenosis.

Splenectomy Does Not Cure the Thrombocytopenia of Systemic Lupus Erythematosus

Fourteen patients with systemic lupus erythematosus had splenectomies done between 1960 and 1982 for treatment of severe thrombocytopenia. Thrombocytopenia persisted or recurred within 1 month postoperatively in five patients and within 6 months in three others. Three patients had late recurrence (18, 30, and 54 months after splenectomy); in two it was probably related to withdrawal of immunosuppressive agents or corticosteroids. Median lowest platelet count before splenectomy and median platelet count at relapse or failure of splenectomy were both 8000/microL. Only two patients maintained normal platelet counts without need for corticosteroids or other treatment. These results differ from those in patients with idiopathic thrombocytopenic purpura. Other treatments should be tried before splenectomy is done for thrombocytopenia in patients with systemic lupus erythematosus.

Ovarian Cancer and Gangrene of the Digits: Case Report and Review of the Literature

Digital ischemia has been reported with various types of cancer, especially gastrointestinal. It is more common in elderly women than in any other group, and the most common symptom is a gangrenous finger (or fingers). More than half of the patients have metastatic involvement. Once the primary disease has been treated, when feasible, the digital symptoms usually regress or disappear. The presence of digital ischemia without other rheumatologic stigmata or vascular predisposition in an elderly patient should raise clinical suspicion of a paraneoplastic phenomenon. Herein we describe a 65-year old woman with digital ischemia associated with ovarian cancer. The diagnosis was established by biopsy after extremely high levels of cancer antigen 125 were detected.

Echovirus polymyositis in patients with hypogammaglobulinemia: Failure of high-dose intravenous gammaglobulin therapy and review of the literature

A 29-year-old man with X-linked hypogammaglobulinemia was treated with prednisone and methotrexate for polymyositis. Subsequently, it was established that disseminated echovirus 11 infection was causing the polymyositis. Treatment with large doses of intravenous gammaglobulin did not result in improvement. Viral cultures of blood, urine, and cerebrospinal fluid gave positive results throughout treatment and at postmortem examination. Multiple cultures of other tissues, including muscle, also gave positive results at postmortem examination. Severity of infection and treatment with prednisone and methotrexate prior to referral, diagnosis, and gammaglobulin treatment may explain the lack of response. A review of 23 cases of echovirus infection in patients with hypogammaglobulinemia revealed that the infection in these patients may cause meningoencephalitis or a polymyositis-like syndrome or both. Treatment with immunosuppressive agents, the standard therapy for polymyositis, is contraindicated, and intravenous or intraventricular gammaglobulin or both may be helpful.