Coronary periarteritis in a patient with multi-organ IgG4-related disease.

Immunoglobulin G4-related disease is a recently described systemic clinicopathological entity characterized by immunoglobulin G4-producing plasmacytic infiltration of tissue and frequently by elevated serum immunoglobulin G4 concentration. Manifestations of this disease have been documented in nearly all organs and locations, but coronary artery involvement is not widely recognized. We report the coronary findings of a patient with multi-organ immunoglobulin G4-related disease. Non-electrocardiogram-gated computed tomography of the chest demonstrated nodular and rind-like periarterial soft tissue thickening along the proximal coronary artery segments with improvement following steroid therapy.

Risk of nephrogenic systemic fibrosis is low in patients with chronic liver disease exposed to gadolinium-based contrast agents.

PURPOSE: To determine the risk of nephrogenic systemic fibrosis (NSF) in a cohort of patients with chronic liver disease. MATERIALS AND METHODS: This retrospective, Institutional Review Board (IRB)-approved, Health Insurance Portability and Accountability Act (HIPAA)-compliant study was performed at a single tertiary liver center. The study cohort comprised 1167 patients with chronic liver disease followed in a liver clinic and exposed to gadolinium-based contrast agents (GBCAs) between February 2004 and October 2007. A retrospective review of medical records was performed. For each patient, data were collected on demographics, history of GBCA exposure, presence of purported risk factors for NSF, and histopathological evidence of NSF. RESULTS: Of the 1167 patients with chronic liver disease, 58% (n = 678) had cirrhosis. The patients had a total of 2421 separate GBCA exposures. Fifty-five percent (n = 646) had a single exposure, 19% (n = 218) had two exposures, and 26% (n = 303) had three or more exposures. Seventy-two percent (n = 843) of patients had renal insufficiency, 25 patients (2.1%) had hepatorenal syndrome, 80 patients (6.8%) were in the perioperative liver transplant period, and 49 patients (4.2%) had one or more additional risk factors for NSF. None of the 1167 patients developed NSF. CONCLUSION: Chronic liver disease does not appear to be a significant risk factor for NSF.

Outbreak of nontuberculous mycobacterial disease in the central Pacific.

Approximately 10% of the island population of Satowan (population, 650 persons), a small, remote coral island in the central Pacific, suffers from an acquired, chronic, disfiguring skin condition known locally as "spam." This skin disease has affected the island population since shortly after World War II. An investigation in 2007 revealed that this skin disease is caused by a nontuberculous mycobacterial infection closely related to Mycobacterium marinum. This article reviews the fascinating history of this skin disease on Satowan, its distinctive clinical presentation, and recommendations for diagnosis and treatment of clinically similar skin lesions in Pacific Islanders.

Sequelae of World War II: an outbreak of chronic cutaneous nontuberculous mycobacterial infection among Satowanese islanders.

BACKGROUND: After World War II, residents of Satowan (population, 650 persons), an outer island in the state of Chuuk, Federated States of Micronesia, noted a high prevalence of a chronic, progressive skin disease known locally as "spam." METHODS: Island residents who had chronic, progressive verrucous or keloidal plaques for >3 months were considered case patients. Tissue specimens were obtained for culture, histopathological analysis, mycobacterial polymerase chain reaction (PCR), and comparison with the hsp65 gene of Mycobacterium marinum. We performed a case-control study involving all cases and randomly selected control individuals from the community. RESULTS: A total of 39 case patients were identified, with a median age of 26.0 years (range, 8-82 years); 74.4% were male, and the mean duration of disease was 12.5 years. A total of 98 control individuals were enrolled. Results of all 19 mycobacterial tissue cultures were negative, and histopathological analysis of all 9 lesions showed suppurative granulomatous inflammation with negative results of mycobacterial and fungal stains. In 7 of 9 paraffin-embedded samples, nontuberculous mycobacterial DNA was detected by PCR, and 2 sequenced products had 95% and 87% identity to M. marinum. All case patients were taro farmers (odds ratio, undefined; P < .01), and among taro farmers, when the analysis was controlled for sex, contact with water-filled World War II-era bomb craters was associated with infection (odds ratio, 8.2; P < .01). CONCLUSIONS: "Spam disease" is a chronic, progressive skin disease of high prevalence on Satowan and is associated with taro farming and contact with World War II-era bomb craters. Histopathological and PCR data demonstrate a nontuberculous mycobacterial infection as the cause.