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R A DeRemee

Publications and source records attributed to R A DeRemee.

At least 55 records · Page 3Linked to original sources
PubMed1990

Clinical implications of the histopathologic diagnosis of pulmonary lymphomatoid granulomatosis.

We reviewed the epidemiologic, laboratory, roentgenographic, pulmonary function, and survival data from 28 patients who had a histologic diagnosis of lymphomatoid granulomatosis (LG) with involvement of the lungs. The mean age at the time of diagnosis was 51 years, and the male-to-female ratio was 3:2. Ten patients had other underlying diseases before LG was diagnosed. The most prominent symptoms were cough, dyspnea, fever, and rash, which were usually present for several months before diagnosis of LG. Multiple nodules were detected on a chest roentgenogram in 68% of the patients. Immunoglobulin concentrations were abnormal in 8 of 12 patients studied. Although bronchoscopy established the diagnosis in approximately a third of the patients who underwent this procedure, open-lung biopsy was uniformly diagnostic. The median survival was 72 months, with follow-up through 12 years. In 11 patients, the original diagnosis of LG was eventually changed to lymphoma. In five of these patients, the change in diagnosis was based on immunohistologic data obtained shortly after LG was discovered. Lymphoma diagnosed in this way was associated with a better prognosis than lymphoma diagnosed on the basis of conventional histopathologic findings. In three patients, solid tumors eventually developed. The diversity of clinical outcomes and frequent revisions of the diagnosis led us to consider the possibility that LG may also represent a histopathologic finding that occurs transiently in several disease processes.

Adult↗
PubMed1990

Granulomatous vasculitis. Wegener's granulomatosis and Churg-Strauss syndrome.

Wegener's granulomatosis and the Churg-Strauss syndrome are both syndromes that appear to begin with a phase of regionally limited symptomatology before they progress at unpredictable rate to a generalized phase characterized by symptoms of systemic vasculitis. The clinical features of atopy, peripheral blood eosinophilia, and tissue eosinophilia distinguish CSS from WG, with its typical necrotizing granulomatous respiratory tract lesions. Whereas in generalized WG with renal involvement the use of cyclophosphamide usually cannot be avoided, the generalized systemic vasculitis phase of CSS appears to respond well to glucocorticoids alone. For the more limited forms of WG, adapted therapy regimens including trimethoprim-sulfamethoxazole have been reported to be successful. Anticytoplasmic autoantibodies (c-ANCA = ACPA) are a new diagnostic serum test with high specificity for WG. Serial determinations of c-ANCA are a promising tool to monitor disease activity.

Churg-Strauss Syndrome↗
PubMed1989

Anticytoplasmic autoantibodies: their immunodiagnostic value in Wegener granulomatosis.

STUDY OBJECTIVE: To determine disease specificity and sensitivity of anticytoplasmic autoantibodies (ACPA) for Wegener granulomatosis, as well as their value as a marker of disease activity. DESIGN: Blind analysis of serum samples, retrospective analysis of clinical data on patients, and prospective follow-up of a subgroup of patients with Wegener granulomatosis. PATIENTS: The study included 277 patients with Wegener granulomatosis (222 with biopsy-proven disease) and 1657 control patients. SETTING: University hospital and academic medical center. LABORATORY INVESTIGATIONS: Analysis of 2653 serum samples from 1934 patients for ACPA. Antibody detection was by indirect immunofluorescence and a new type of enzyme-linked immunoadsorbent assay (ELISA). Prospective follow-up was on 172 patients with Wegener granulomatosis. MEASUREMENTS AND MAIN RESULTS: Specificity of ACPA for Wegener granulomatosis measured by indirect immunofluorescence was 99% (CI, 98.9% to 99.7%) and 98% (CI, 97.4% to 99.2%) by ELISA. Sensitivity of ACPA depended on disease activity and extent: It was 67% (CI, 38% to 89%) by immunofluorescence and 60% (CI, 32% to 84%) by ELISA for patients with active locoregional symptomatology (n = 15); and 32% (CI, 14% to 54%) by immunofluorescence and 40% (CI, 21% to 61%) by ELISA for patients in full remission after initial locoregional symptoms (n = 25). The sensitivity was 96% (CI, 89% to 99%) by immunofluorescence and 93% (CI, 86% to 98%) by ELISA for patients with active generalized disease (n = 92). Serial testing was done; every patient with active generalized disease eventually had at least one positive serum sample. Sensitivity decreased to 41% (CI, 22% to 62%) by both immunofluorescence and ELISA for patients in full remission after active generalized disease (n = 27). Levels of ACPA expressed both as immunofluorescence titers and ELISA values (U/mL) correlated well with disease activity. CONCLUSIONS: Testing for ACPA in serum of patients with Wegener granulomatosis is valuable for differential diagnosis; furthermore, APCA can be used as a marker to follow disease activity. A new type of ELISA yielded the same results as indirect immunofluorescence for the specificity, sensitivity, and correlation with disease activity of ACPA.

Adolescent↗
PubMed1989

Two-dimensional echocardiographic findings in systemic sarcoidosis.

The records of 88 patients with systemic sarcoidosis who underwent 2-dimensional echocardiography were reviewed to define the typical echocardiographic abnormalities in patients with clinical evidence of cardiac involvement. Twelve patients (14%) had echocardiographic evidence of left ventricular systolic dysfunction attributed to cardiac sarcoidosis (segmental hypokinesis in 8 patients and global hypokinesis in 4 patients). A high incidence of congestive heart failure (9 of 12) and conduction system disease (7 of 12) was characteristic of these patients. The remaining 76 patients had normal echocardiograms or abnormalities attributed to nonsarcoid sources. In 8 (11%) of these 76 patients, there were significant clinically unexplained conduction abnormalities likely related to sarcoidosis.

Adult↗
PubMed1989

Anticytoplasmic autoantibodies in the diagnosis and follow-up of Wegener's granulomatosis.

Sixty-five patients with biopsy-proven Wegener's granulomatosis (WG), 54 with systemic vasculitis, 22 with relapsing polychondritis, 20 with sarcoidosis, 20 with malignant pulmonary lesions, and 15 with other conditions underwent determination of anticytoplasmic autoantibodies (ACPA) by the indirect immunofluorescence technique on neutrophil cytospin preparations to assess the specificity of ACPA for WG, their sensitivity in relationship to the extent and activity of the disease, and their value for follow-up of WG. Of these 65 patients with WG, 38 were ACPA positive. Two patients in the vasculitis group, best categorized as having microscopic polyarteritis, were ACPA positive. We obtained 125 serum samples from the 65 patients with WG and assigned them to one of two categories (limited or generalized), based on the extent of disease. Each of these categories was then subdivided into "active" or "in remission." Median ACPA titers were significantly different between active disease and remission in each category, as well as between active limited and active generalized disease. All patients whose disease changed from active to in remission had reductions in ACPA titer levels; those who experienced flares had titer increases. Patients with intercurrent illnesses or complications of treatment, mimicking WG flares, did not have titer increases. We conclude that ACPA determined by the indirect immunofluorescence technique is highly specific for WG. The sensitivity is dependent on the extent and activity of WG, and serial titer determinations are valuable in monitoring disease activity.

Adolescent↗
PubMed1988

Cyclophosphamide-induced bladder toxicity in Wegener's granulomatosis.

In a series of 111 patients with Wegener's granulomatosis who were given cyclophosphamide therapy, hemorrhagic cystitis, diagnosed on the basis of gross hematuria or at cystoscopy (or both), developed in 17 (15%). Most of these patients recovered uneventfully, with or without the discontinuation of cyclophosphamide, but 4 patients suffered a significant loss of blood, and bladder carcinoma developed in 3. New microhematuria also occurred in 52 patients (47%). The dose and duration of cyclophosphamide were greater in the group that had urotoxicity. Long-term followup of patients with hemorrhagic cystitis is mandatory for the detection of late recurrences or the development of bladder malignancy. New therapies are being directed at protecting the bladder from urotoxicity during cyclophosphamide treatment.

Administration, Oral↗
PubMed1988

The influence of diagnostic access bias on the epidemiology of sarcoidosis: a population-based study in Rochester, Minnesota, 1935-1984.

A population-based study was conducted among residents of Rochester, Minnesota from 1935-1984 to examine the influence of diagnostic access bias on the incidence and mode of presentation of clinically recognized sarcoidosis. Immigrants had an elevated risk of sarcoidosis and were more likely to present with asymptomatic sarcoidosis diagnosed on routine screening chest radiographs. Similar patterns were observed for health professionals, a group with markedly greater access to the diagnostic procedures which label them as having sarcoidosis. Sarcoidosis exhibits a spectrum of manifestations, ranging from a totally asymptomatic process discernible only by specific diagnostic scrutiny to a clinically obvious presentation with significant morbidity. For disease entities such as this, apparent patterns of occurrence may depend on differential access in the study population to the maneuvers necessary for diagnosis. Diagnostic access bias, in addition to other diagnostic biases, deserves more careful scrutiny in epidemiologic research.

Adolescent↗
PubMed1988

Benign lymphocytic angiitis and granulomatosis: experience with three cases.

Of three patients with lung-biopsy-proven benign lymphocytic angiitis and granulomatosis, two experienced complete resolution of their disease from therapy with chlorambucil and one underwent spontaneous remission with no drug therapy after lung biopsy and removal of a benign spindle cell thymoma. Clinically, it is difficult to determine whether benign lymphocytic angiitis is a low-grade (prelymphomatous) lymphoma or a vasculitis. Its position in this spectrum of diseases is uncertain. Nonetheless, benign lymphocytic angiitis and granulomatosis corresponding to a low-grade angiocentric immunoproliferative lesion is a clinicopathologically useful concept.

Aged↗
PubMed1987

Prostatic involvement in Wegener's granulomatosis.

Wegener's granulomatosis involving the prostate gland is unusual. We report 3 cases of this condition in which typical necrotizing granulomas with vasculitis were seen histologically. These lesions may cause gross hematuria or obstructive voiding, including urinary retention. Management includes prostatectomy when the symptoms are severe but an initial trial of aggressive medical therapy may be successful. Treatment requires a knowledge of the natural history of Wegener's granulomatosis and its usual response to medical therapy.

Aged↗
PubMed1987

Diffuse interstitial pulmonary disease from the perspective of the clinician.

Diffuse interstitial pulmonary disease encompasses a large number of diverse entities having in common the diffuse infiltration of alveolar walls by inflammatory or malignant cells, connective tissue, or fluid that impairs transfer of oxygen to blood. Pertinent clues to the diagnosis may be obtained by careful analysis of history, physical examination, chest roentgenogram, pulmonary function, and other laboratory data. This analytic process is referred to as the "algorithmic synthesis." This synthesis can guide the clinician in the selection of tissue sampling techniques most likely to confirm the diagnosis.

Algorithms↗
PubMed1985

Serum angiotensin-converting enzyme activity. Its use in the evaluation and management of hypercalcemia associated with sarcoidosis.

Serum angiotensin-converting enzyme (SACE) was measured in 14 patients (eight women and six men) with sarcoidosis and hypercalcemia. Thirteen patients were treated with prednisone, and 12 achieved normal or nearly normal serum calcium values. Two patients had coexistent hyperparathyroidism. Seven of eight patients with serial SACE measurements exhibited parallel falls in SACE and serum calcium levels. Eleven patients were successfully treated with alternate-day prednisone regimens. The data suggest that serial SACE measurements are useful in the evaluation and management of sarcoidosis with hypercalcemia. In patients with sarcoidosis, the reduction of SACE levels during glucocorticoid treatment may be due to a suppression of granuloma formation. Concomitant falls in serum calcium level suggest an important role of the granuloma or its cellular precursors in vitamin D metabolism.

Adult↗