Racial differences in sarcoidosis granuloma density.

STUDY OBJECTIVES: While sarcoidosis generally inflicts a greater morbidity on African-American compared with Caucasian patients, no studies have examined whether racial differences exist in the intensity of the histologic hallmark of sarcoidosis, noncaseating granulomas. DESIGN AND SETTING: The study was conducted as a retrospective case series in a tertiary referral center. PATIENTS: The study included 187 patients with histopathologic confirmation of sarcoidosis by trans- and/or endobronchial biopsy between July 1991 and December 2001. MEASUREMENTS AND RESULTS: Granuloma density was the average number of granulomas per biopsy piece on the slide with the most intense granulomatous inflammation at fourfold magnification. Overall, African-American patients had a twofold greater median granuloma density than Caucasians (p = 0.005). In a negative binomial multivariate model, radiographic pattern had the strongest association with granuloma density, with Scadding stage II and III patients having adjusted granuloma densities of 60% (p = 0.005) and 105% (p = 0.0001) higher than stage I patients. In the specific-tissue types, radiographic stage-adjusted granuloma densities in African-American patients were 49% greater in bronchial tissue (p = 0.03), but only a 27% greater in alveolar tissue (p = 0.51). CONCLUSIONS: A greater granuloma density in bronchiolar lung tissue of African-American sarcoidosis patients may explain racial differences in diagnostic yield by lung biopsy and disease severity at diagnosis. This association persists even after controlling for Scadding radiographic stage, a measure of disease severity strongly associated with granuloma density.

Differences in clinical findings between Caucasians and African Americans with biopsy-proven sarcoidosis.

OBJECTIVE: To study the prevalence of ocular manifestations in African American and Caucasian patients with biopsy-proven sarcoidosis at the initial ophthalmic examination and to determine the relationship between angiotensin-converting enzyme (ACE) levels, chest x-ray findings, and ocular signs of sarcoidosis. DESIGN: Retrospective, cross-sectional, observational study. PARTICIPANTS: Eighty-one consecutive patients with biopsy-proven sarcoidosis seen at the Doheny Eye Institute from January 1989 through April 2005. METHODS: Medical records were reviewed to obtain demographic data, biopsy site, initial ocular findings, pulmonary symptoms, and results of serum ACE levels and chest x-rays. Associations between ACE level/chest x-ray stages and ocular manifestations related to sarcoidosis were obtained from these data. MAIN OUTCOME MEASURES: Ocular manifestations related to sarcoidosis. RESULTS: Of the 81 patients, 35 were Caucasian; 29 were African American; and the remaining 17 were Hispanic, Asian Indian, and other races. Female patients were older than males (P = 0.05). Sixty-five (80%) of the 81 patients had ocular manifestations related to sarcoidosis. Thirty-three patients (40.7%) had uveitis, 12 (14.8%) had adnexal granulomas, and 25 (30.8%) had keratoconjunctivitis sicca. Of the 33 patients with uveitis, 22 presented with nongranulomatous inflammation. There was no significant association between ocular manifestations related to sarcoidosis and serum ACE levels (P = 0.43) or chest x-ray stage (P>0.99). Of the 29 African American patients, 26 (89.7%) had ocular manifestations related to sarcoidosis, compared with 24 (68.6%) of the 35 Caucasians (P = 0.12). The African American patients were younger (mean age, 44.4 years) than the Caucasian patients (mean age, 52.0) (P = 0.003) and had higher mean ACE levels (P = 0.003). A significantly high proportion of African American males presented with uveitis (P = 0.005), and a significantly high proportion of African American females presented with adnexal granulomas (P = 0.05). CONCLUSIONS: The present study reveals that patients with sarcoidosis can present initially with clinical features of nongranulomatous uveitis. Relative to Caucasians, African American patients with sarcoidosis tend to be younger when they first present to the ophthalmologist and to present with uveitis and/or adnexal granuloma. Serum ACE levels and chest x-ray stages may not help predict the occurrence of ocular changes in sarcoidosis.

A clinicopathological review of 34 cases of inflammatory breast disease showing an association between corynebacteria infection and granulomatous mastitis.

AIM: Granulomatous mastitis is a rare condition of unknown aetiology. The great majority of cases has not been associated with bacterial pathogens if women with mammary tuberculosis are excluded. We noted that some women in Auckland with a histological diagnosis of granulomatous mastitis had both microbiological and histological evidence of corynebacteria infection and aimed to study this further. METHODS: Thirty-four women were reviewed who presented with inflammatory breast disease and had microbiological specimens from which corynebacteria were isolated and/or histological specimens containing coryneform bacteria. These 34 cases were compared with 28 controls with similar histology but no evidence of corynebacteria infection. RESULTS: Twenty-seven (79%) of the cases and 21 (75%) of the controls had histological and/or cytological evidence of suppurative granulomas. Fourteen of the 34 cases also had Gram-positive bacilli (GPB), recognisable as coryneform bacteria, in histological sections. In all cases the bacilli were confined to empty spaces, consistent with dissolved lipid, and were surrounded by neutrophils and, frequently, suppurative granulomas. Corynebacterium species were isolated from 52 of 116 microbiological specimens taken from the 34 cases. Forty of these 52 cultures were pure. Twenty-four of the cultures were further classified biochemically and using 16S rRNA gene sequencing. Twenty of the 24 were lipophilic Corynebacterium species and 14 were identified as Corynebacterium kroppenstedtii. The cases were more likely to present with fever or neutrophilia and more often formed sinuses than the controls but other clinical features were similar. Maori and Pacific Islanders accounted for 77% of the women across both groups. CONCLUSION: We suggest granulomatous mastitis can be associated with corynebacteria infection, particularly infection by C. kroppenstedtii. The significance of this finding, which has previously been described in only a single case report, is discussed.

Childhood granulomatous periorificial dermatitis in an Asian girl--a variant of sarcoid?

Childhood granulomatous periorificial dermatitis (CGPD) is a benign, facial eruption which has previously been reported in prepubertal, Afro-Caribbean children. It is a condition of unknown aetiology, characterized by a monomorphic, papular eruption around the mouth, nose and eyes. Typically the eruption persists for several months but resolves without scarring. The condition can usually be distinguished from sarcoidosis on clinical and histological grounds. To our knowledge, this is the first case of CGPD occurring in an Asian child with sarcoidal histology and is unusual in its persistence for 4 years. We suggest that this case of CGPD may well be a variant of sarcoid.

Familial granulomatous hepatitis: a hitherto unrecognized entity.

Two West Indian parents and three of their seven offspring presented over a 12-yr period with identical systemic illnesses characterized by the development of granulomatous hepatitis. Granulomata were variably also found in muscle, lymph nodes, and pleura in some of these affected individuals. The usual causes of granulomatous hepatitis were absent, and this familial disease represents a hitherto undescribed entity.

Familial granulomatous hepatitis: a hitherto unrecognized entity

Two West Indian parents and three of their seven offspring presented over a 12 yr period with identical systemic illnesses characterized by the development of granulomatous hepatitis. Granulomata were variably also found in muscle, lymph nodes, and pleura in some of these affected individuals. The usual causes of granulomatous hepatitis were absent, and this familial disease represents a hitherto undescribed entity. (AU)