PubMed Health⌕ Search

Biomedical subjects

R P Baughman

Publications and source records attributed to R P Baughman.

At least 19 recordsLinked to original sources
PubMed2006

Presenting characteristics as predictors of duration of treatment in sarcoidosis.

BACKGROUND: Some sarcoidosis patients never need therapy, but many still require therapy more than 2 years after initial diagnosis. AIM: To determine what features at initial presentation are associated with treatment 2 years later. METHODS: Patients with biopsy-confirmed sarcoidosis enrolled in the ACCESS (A Case Control Etiologic Study of Sarcoidosis) study were initially evaluated within 6 months of diagnosis. Pulmonary function, chest X-ray and dyspnoea score were measured, and systemic therapy for the sarcoidosis recorded. Organ involvement was assessed using a standardized instrument. A subset (n = 215) were seen 18-24 months later for follow-up, and these patients constitute our study group. RESULTS: Ten patients had only received therapy before the first visit, with no further therapy, and were excluded from analysis. Of the remaining 205, 95 were not on therapy at the initial visit and 75 (79%) of these were never treated during follow-up. Of the 110 initially on therapy, 52 (47%) remained on therapy at follow-up. Other initial features associated with continued therapy were the level of dyspnoea and predicted vital capacity. On logistic regression, only dyspnoea and therapy at initial visit remained significant. Patients on systemic therapy at initial evaluation were more likely to be on therapy at follow-up (OR 3.6, p = 0.003). Neither ethnicity nor gender independently predicted therapy at follow-up. DISCUSSION: This study group represents a sample of newly diagnosed sarcoidosis patients. However, this is a referral population, and there was no set protocol for treatment. Use of systemic therapy within the first 6 months after diagnosis appears to be strongly associated with continued use of therapy 2 years later.

Adult↗
PubMed2005

Fungal infections as a complication of therapy for sarcoidosis.

BACKGROUND: Treatment of symptomatic sarcoidosis usually includes systemic immunosuppressive agents. These agents may render the patient more susceptible to opportunistic infections. In addition, the fungal infection may be difficult to distinguish from the underlying sarcoidosis. AIM: To examine the presentation and management of invasive fungal infections in sarcoidosis patients. DESIGN: Retrospective record review. METHODS: We reviewed the notes of all sarcoidosis patients (n = 753) seen at our clinic over an 18-month period. RESULTS: Seven patients (0.9%) with previously diagnosed sarcoidosis developed fungal infections: two each with Histoplasma capsulatum and Blastomyces dermatitidis and three others with Cryptococcus neoformans. No cases of invasive aspergillus or tuberculosis were identified. The diagnosis of fungal infection was made by bronchoscopy (four cases), open-lung biopsy (one case), bone-marrow aspirate (one case), and spinal fluid examination (one case). All patients were receiving corticosteroids at the time of worsening chest X-ray or clinical status. Four patients were also receiving methotrexate prior to infection. No patient with systemic fungal infection was receiving either infliximab or cyclophosphamide. All patients responded to anti-fungal therapy and a reduction in immunosuppression. DISCUSSION: Fungal infections occur rarely in treated patients with sarcoidosis. Deterioration of chest X-ray, especially a localized infiltrate, warrants investigation.

Antifungal Agents↗
PubMed2005

Genome-wide search for sarcoidosis susceptibility genes in African Americans.

Sarcoidosis, a systemic granulomatous disease of unknown etiology, likely results from an environmental insult in a genetically susceptible host. In the US, African Americans are more commonly affected with sarcoidosis and suffer greater morbidity than Caucasians. We searched for sarcoidosis susceptibility loci by conducting a genome-wide, sib pair multipoint linkage analysis in 229 African-American families ascertained through two or more sibs with a history of sarcoidosis. Using the Haseman-Elston regression technique, linkage peaks with P-values less than 0.05 were identified on chromosomes 1p22, 2p25, 5p15-13, 5q11, 5q35, 9q34, 11p15 and 20q13 with the most prominent peak at D5S2500 on chromosome 5q11 (P=0.0005). We found agreement for linkage with the previously reported genome scan of a German population at chromosomes 1p and 9q. Based on the multiple suggestive regions for linkage found in our study population, it is likely that more than one gene influences sarcoidosis susceptibility in African Americans. Fine mapping of the linked regions, particularly on chromosome 5q, should help to refine linkage signals and guide further sarcoidosis candidate gene investigation.

Black or African American↗
PubMed2005

Infliximab in chronic ocular inflammation.

OBJECTIVE: Infliximab is a chimeric antibody which binds tumor necrosis factor (TNF). It is effective in several chronic inflammatory conditions, including sarcoidosis. METHODS: We report our experience with infliximab in chronic ocular inflammation as part of a retrospective review of all patients treated for chronic inflammatory ocular conditions seen over a 2-year period at our institution. RESULTS: 14 patients with various underlying ocular conditions were treated during the previous two years including patients with sarcoidosis (7), Crohn's disease (2), birdshot choroiditis (2), idiopathic disease (2), Volt-Koyanagi-Harada (1) and Behçet's disease (1). All patients had persistent inflammation despite systemic immunosuppressive agents and all but one patient experienced marked improvement in ocular inflammation with infliximab. One patient was non-compliant and non-evaluable; four patients, who had previously received etanercept with either no response (3 patients) or subsequent relapse (1 patient), responded to infliximab. CONCLUSION: Infliximab is an effective therapy in chronic inflammatory eye disease, especially when related to sarcoidosis.

Adult↗
PubMed2003

Difficult treatment issues in sarcoidosis.

The management of sarcoidosis includes several crucial decisions. Not all patients with sarcoidosis need treatment. At least a third of patients will never be treated. It is unclear whether asymptomatic patients ever need therapy, even if they have extensive lung disease. One reason that clinicians are reluctant to start therapy is that many patients who are started on corticosteroids have a difficult time getting off therapy, even after 2 years. In the chronic patient, alternatives to corticosteroids have been developed. These include drugs such as methotrexate, azathioprine and hydroxychloroquine. These agents have been the standard second line of therapy for patients with chronic disease. However, these drugs do not always work. In addition, they are associated with their own toxicities. Another group of sarcoidosis patients have also emerged. These are the refractory patients, who have progressive disease whilst on therapy. For these patients, new agents such as thalidomide and the monoclonal antibodies to tumour necrosis factor have been occasionally helpful. This paper reviews several important issues in the management of sarcoidosis.

Antibodies, Monoclonal↗
PubMed2001

Familial aggregation of sarcoidosis. A case-control etiologic study of sarcoidosis (ACCESS).

Despite reports of familial clustering of sarcoidosis, little empirical evidence exists that disease risk in family members of sarcoidosis cases is greater than that in the general population. To address this question, we estimated sarcoidosis familial relative risk using data on disease occurrence in 10,862 first- and 17,047 second-degree relatives of 706 age, sex, race, and geographically matched cases and controls who participated in the multicenter ACCESS (A Case-Control Etiology Study of Sarcoidosis) study from 1996 to 1999. Familial relative risk estimates were calculated using a logistic regression technique that accounted for the dependence between relatives. Sibs had the highest relative risk (odds ratio [OR] = 5.8; 95% confidence interval [CI] = 2.1-15.9), followed by avuncular relationships (OR = 5.7; 95% CI = 1.6-20.7), grandparents (OR = 5.2; 95% CI = 1.5-18.0), and then parents (OR = 3.8; 95% CI = 1.2-11.3). In a multivariate model fit to the parents and sibs data, the familial relative risk adjusted for age, sex, relative class, and shared environment was 4.7 (95% CI = 2.3-9.7). White cases had a markedly higher familial relative risk compared with African-American cases (18.0 versus 2.8; p = 0.098). In summary, a significant elevated risk of sarcoidosis was observed among first- and second-degree relatives of sarcoidosis cases compared with relatives of matched control subjects.

Adolescent↗
PubMed2001

Clinical characteristics of patients in a case control study of sarcoidosis.

Sarcoidosis may be affected by sex, race, and age. A Case Control Etiologic Study of Sarcoidosis (ACCESS) enrolled 736 patients with sarcoidosis within 6 mo of diagnosis from 10 clinical centers in the United States. Using the ACCESS sarcoidosis assessment system, we determined organ involvement for the whole group and for subgroups differentiated by sex, race, and age (less than 40 yr or 40 yr and older). The study population was heterogeneous in terms of race (53% white, 44% black), sex (64% female, 36% male), and age (46% < 40 yr old, 54% > or = 40 yr old). Women were more likely to have eye and neurologic involvement (chi(2) = 4.74, p < 0.05 and chi(2) = 4.60, p < 0.05 respectively), have erythema nodosum (chi(2) = 7.28, p < 0.01), and to be age 40 yr or over (chi(2) = 6.07, p < 0.02) whereas men were more likely to be hypercalcemic (chi(2) = 7.38, p < 0.01). Black subjects were more likely to have skin involvement other than erythema nodosum (chi(2) = 5.47, p < 0.05), and eye (chi(2) = 13.8, p < 0.0001), liver (chi(2) = 23.3, p < 0.0001), bone marrow (chi(2) = 18.8, p < 0.001), and extrathoracic lymph node involvement (chi(2) = 7.21, p < 0.01). We conclude that the initial presentation of sarcoidosis is related to sex, race, and age.

Adult↗
PubMed2001

Role of bronchoalveolar lavage in interstitial lung disease.

Bronchoalveolar lavage remains an important research tool in understanding ILD. It is still an important part of the clinical management of patients with ILD. It is most useful in detecting unusual forms of ILD. It helps the clinician narrow down the possible causes of the interstitial pattern. It also can confirm a clinical impression of certain conditions. Although rarely diagnostic, it is often supportive. In conjunction with high-resolution CT scan, most patients with ILD can be diagnosed using relatively noninvasive methods.

Bronchoalveolar Lavage Fluid↗
PubMed2001

Surfactant protein deficiency in familial interstitial lung disease.

OBJECTIVE: To determine the contribution of surfactant protein abnormalities to the development of chronic lung injury in a familial form of interstitial lung disease. STUDY DESIGN: An 11-year-old girl, her sister, and their mother who were diagnosed with chronic interstitial lung disease underwent laboratory investigation of surfactant protein expression in bronchoalveolar lavage fluid and lung biopsy specimens. Nineteen patients with idiopathic pulmonary fibrosis and 9 patients who were investigated for pulmonary malignancy but who did not have interstitial lung disease served as control subjects. RESULTS: The 3 family members were found to have absent surfactant protein C (SP-C) and decreased levels of SP-A and SP-B in bronchoalveolar lavage fluid (BALF). Immunostaining for pulmonary surfactant proteins in lung biopsy specimens obtained from both children demonstrated a marked decrease of pro-SP-C in the alveolar epithelial cells but strong staining for pro-SP-B, SP-B, SP-A, and SP-D. No deviations from published surfactant protein B or C coding sequences were identified by DNA sequence analysis. All control subjects had a detectable level of SP-C in the BALF. CONCLUSION: The apparent absence of SP-C and a decrease in the levels of SP-A and SP-B are associated with familial interstitial lung disease.

Adult↗
PubMed2001

Nonsteroidal therapy for idiopathic pulmonary fibrosis.

The treatment of interstitial lung disease usually has consisted of corticosteroids with or without either azathioprine or cyclophosphamide. These drugs mainly target the neutrophil. Recently, the role of the Th2 lymphocyte leading to fibrosis has been demonstrated in animal models and preliminary human studies. This finding has led to interest in cytokine therapy with the interferon treatments, which reduce the Th2 response. Other new treatments have focused specifically on the fibroblast or oxygen free radicals. The range of treatment for interstitial lung diseases is increasing. Current clinical trials are in progress to confirm the pilot studies recently reported. The future therapy of interstitial lung disease probably will consist of multiple agents aimed at several aspects of the inflammatory reaction of interstitial lung disease.

Azathioprine↗
PubMed2001

Depression in sarcoidosis.

Sarcoidosis, a chronic, multisystem disease, impacts quality of life and may increase depression risk. No previous study has reported the depression prevalence among U.S. sarcoid patients. This cross-sectional study examined sociodemographic and disease morbidity factors associated with depression. Patients diagnosed for > or = 1 yr and treated at one of six centers were eligible (n = 176); 154 completed a questionnaire of demographics, treatment, access to medical care, and a short-form Center for Epidemiologic Studies- Depression Scale (CES-D). The primary outcome variable was a CES-D score of > or = 9, indicating clinical depression. The prevalence of depression was 60%. Gender, income, access to medical care, dyspnea on exertion, and number of systems involved were associated with depression. Female sex, decreased access to medical care, and increased dyspnea predicted depression (odds ratio [OR] = 3.33, 11.64, and 2.78, respectively) after adjusting for race, income, and steroid therapy. Despite tertiary care access, patients reported medical care limitation. Health care providers must be sensitive to multiple barriers faced by chronic sarcoid patients; acknowledging depression risk and improving access to medical care will promote better overall health among sarcoid patients. Future studies of sarcoidosis will need to address depression diagnosis and treatment.

Adult↗
PubMed2001

Infliximab for refractory sarcoidosis.

BACKGROUND AND AIM OF WORK: Tumor necrosis factor-alpha (TNF-alpha) appears to be an important cytokine in the inflammation of sarcoidosis. Infliximab is a chimeric monoclonal antibody which specifically inhibits TNF-alpha. We investigated the efficacy of infliximab for the therapy of chronic, resistant sarcoidosis. METHODS: Patients with persistent symptomatic sarcoidosis despite corticosteroids and immunosuppressive agents were selected for treatment with infliximab. Patients were treated initially and at 2, 4, and 12 weeks with 5 mg/kg of infliximab at each treatment. Index lesions, which had progressed despite corticosteroid therapy, were reevaluated at 16 weeks. RESULTS: Three patients were treated. In two patients, the index lesion was lupus pernio, which significantly improved with infliximab. The third patient had restrictive lung disease. At week 16, there was a 26% improvement in the vital capacity from pretreatment values. All patients tolerated the treatments well. CONCLUSIONS: Infliximab was associated with significant improvement in chronic sarcoidosis.

Antibodies, Monoclonal↗
PubMed2001

Combination therapy for sarcoidosis.

Combination therapy has proved useful in infectious, rheumatologic, and oncologic diseases. The role of combination therapy in sarcoidosis is less defined. A stepwise approach to therapy in sarcoidosis treatment includes multiple agents, such as topical and systemic corticosteroids. The introduction of cytotoxic agents has led to the combination of these drugs with lowered doses of corticosteroids. Recently, the combination of cytotoxic and immune modifiers has been used for some cases of refractory sarcoidosis. The rationale use of combination therapy may enhance efficacy with reduced toxicity.

Adrenal Cortex Hormones↗
PubMed2001

Breast disease in sarcoidosis.

BACKGROUND: Breast disease in sarcoidosis can be classified as sarcoidosis patients with breast granulomas, sarcoidosis patients with breast cancer, and breast cancer patients displaying sarcoidosis-like breast reactions. METHODS: We reviewed the medical records of 629 women with sarcoidosis followed in the Interstitial Lung Disease Clinic at the University of Cincinnati for findings associated with breast disease. In addition, three women with breast cancer who had granulomas in proximity to their tumors were also examined. RESULTS: Abnormal breast examinations or mammograms were reported in 15 patients with sarcoidosis (2% of women with sarcoidosis). Breast biopsy revealed granulomas consistent with sarcoidosis in six. One of them developed breast cancer five years later. Breast cancer was identified in twelve further patients, therefore a total of thirteen patients with breast cancer were identified. Ten were diagnosed with breast cancer plus sarcoidosis: sarcoidosis preceded breast cancer in three, followed breast cancer in five, the two diseases appeared simultaneously in two. Three additional women with breast cancer were also evaluated and classified as patients with sarcoid-like reaction. Review of the mammographic and physical findings could not distinguish between sarcoidosis in the breast and breast cancer. CONCLUSION: Sarcoidosis patients develop breast cancer at the expected frequency. The breast cancer diagnosis may precede or follow that of sarcoidosis. There is no relationship between stage of sarcoidosis or treatment and the development of cancer. Because physical examination and mammography findings are unable to distinguish between sarcoidosis and malignancy, biopsy of all suspicious lesions in sarcoidosis is recommended.

Adrenal Cortex Hormones↗
PubMed2000

Ventilator associated pneumonia: quality of nonbronchoscopic bronchoalveolar lavage sample affects diagnostic yield.

The importance of predefined criteria for acceptable samples of respiratory therapists obtained lower respiratory samples were studied, using a nonbronchoscopic bronchoalveolar lavage (BAL) protocol for ventilated patients in the intensive care unit. Therapists were instructed and asked to follow guidelines for obtaining samples. Over one year, 219 samples were obtained by respiratory therapists. Of these, 115 were considered to be adequate samples using the following criteria: 60 mL of instilled volume, at least 5 mL of fluid aspirated, specimens sent for semiquantitative culture, a differential cell count of <5% bronchial epithelial cells. Overall, 52 samples grew one or more pathogen at >10,000 colony forming units (cfu).mL(-1) of BAL. The most common pathogen was Staphylococcus aureus (S. aureus) (11 samples), although Gram-negative bacilli were the single pathogen in 21 specimens. Of the 115 acceptable samples, 40 (35%) grew > or =1 pathogen at >10,000 cfu.mL(-1). For the 80 not acceptable samples which were sent for appropriate culture, 12 (15%) grew >10,000 cfu.mL(-1) BAL. This difference was significant (Chi-squared=9.44, p<0.01). Nonbronchoscopic bronchoalveolar lavage can be safely performed by respiratory therapists'. The authors recommend that a protocol be used to evaluate the quality of a bronchoalveolar lavage sample in the same manner sputum samples are screened prior to interpretation.

Bronchoalveolar Lavage Fluid↗
PubMed2000

Infections in acute exacerbation of chronic bronchitis: what are they and how do we know?

Acute exacerbations of chronic bronchitis can be due to many factors. The most commonly recognized factor is infection, with bacterial infection being identified in about fifty percent of patients with chronic bronchitis. The causative agent can be detected by several different methods. The most common is sputum examination; however, more invasive techniques have been studied. These include transtracheal aspirates and bronchoscopic samples. The most widely studied bronchoscopic sample has been the protected brush specimen (PBS). Despite the wide array of sampling techniques, the pathogens which have been identified have been relatively consistent. The three most common pathogens have been H. influenzae, S. Pneumoniae, and B. catarrhalis. Other pathogens, including gram negative enteric organisms, are seen in patients with more advanced disease. The information obtained by the diagnostic studies has allowed clinicians to develop treatment strategies for AECB. These diagnostic studies will be needed to help upgrade treatment guidelines as new bacteria and bacterial resistance patterns change.

Journal Article↗
PubMed2000

Geographic clustering of Pneumocystis carinii pneumonia in patients with HIV infection.

To detect whether there was geographic clustering of Pneumocystis carinii pneumonia cases among patients with human immunodeficiency virus (HIV) infection, we performed a retrospective analysis of a clinical database. The rates of pneumocystosis were analyzed by zip code zones for evidence of geographical clustering. During the study period, 118 patients at our AIDS Treatment Center had a first episode of P. carinii pneumonia. An analysis of the 24 zip code zones for which a P. carinii pneumonia rate was calculated (requiring a denominator of at least 10 known HIV- infected individuals residing in that zone) showed a trend toward geographic clustering (p = 0.07); when all 45 Cincinnati zip code zones were included in the analysis, clustering of cases was observed (p = 0. 02). By contrast, no clustering was observed for 52 HIV-infected control subjects with respiratory disease or for 960 HIV-infected patients treated at our center during the same time period. These data raise intriguing questions about exposure to exogenous sources of P. carinii and suggest the need for prospective studies.

AIDS-Related Opportunistic Infections↗