The organ-specific extrapulmonary presentation of sarcoidosis: a frequent occurrence but a challenge to an early diagnosis. A 3-year-long prospective observational study.

BACKGROUND AND AIM: The percentage of organ-specific extrapulmonary presentation of sarcoidosis has usually been estimated on the basis of retrospective studies, with figures varying in the range of 7-22%. We have hypothesized that a prospective study could give a higher figure, and could better outline the variable pattern of extrapulmonary presentation.

MATERIAL AND

METHODS

A careful examination with particular interest to the possible extrapulmonary signs or symptoms of the disease, even if previously unrecognized, was carried out in 204 consecutive sarcoid patients (mean age 37 +/- 11) seen between 2000 and 2002. Some of them had already visited our clinic in previous years. Their median follow up in our clinic was 34 months, while the median duration of disease was 60 months.

RESULTS

An organ-specific extrapulmonary presentation did occur in 73 patients (35.78%). The most frequent extrapulmonary presentation was due to skin lesions (different from erythema nodosum) and to peripheral lymph nodes (8% each). Less frequent were renal stones (4%), uveitis (3%) and others (12%). The extrapulmonary presentation may be a challenge to the diagnosis. In our series an early diagnosis was reached only in 38 of 73 patients. In the other 35 patients, the diagnosis was reached later, after a time interval ranging from 6 months to 20 years. The lung involvement was detected early in 33 patients with extrapulmonary presentation. In 35 other patients the pulmonary involvement appeared after a median time interval of 24 months (range 6-240, interquartiles 15-60). In 5 patients, no pulmonary changes ever appeared after a long term follow up of 20 months, 5 years (2 patients), 10 years and 29 years respectively.

CONCLUSION

An organ-specific extrapulmonary presentation is common in sarcoidosis, but may go unrecognized for years.