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Extrapulmonary location of sarcoidosis
I Pokrzywnicka-Gajek1, A Jedryka-Góral, H Chwalińska-Sadowska
1Department of Connective Tissue Diseases, Institute of Rheumatology, Warszawa, Poland.
Materia Medica Polona. Polish Journal of Medicine and Pharmacy
|January 1, 1992
Summary
This study reports two cases of extrapulmonary sarcoidosis presenting with sicca syndrome. Both patients responded well to glucocorticosteroid treatment, indicating effective management of this rare manifestation.
Area of Science:
- Immunology
- Rheumatology
- Pathology
Background:
- Sarcoidosis is a multisystem inflammatory disease of unknown etiology.
- Extrapulmonary sarcoidosis can present with diverse clinical manifestations.
- Sicca syndrome, characterized by dry eyes and dry mouth, can be a prominent feature.
Observation:
- Two patients presented with predominant symptoms of sicca syndrome.
- Case 1 exhibited mediastinal/retroperitoneal lymphadenopathy and lacrimal gland impairment.
- Case 2 showed polyneuropathy and a maculopapular skin rash alongside sicca symptoms.
Findings:
- Histological examination confirmed sarcoidosis via lower lip mucosa biopsy (Case 1) and skin biopsy (Case 2).
- Elevated erythrocyte sedimentation rate and circulating immune complexes were noted in laboratory tests.
- Serological tests for rheumatoid factor, antinuclear antibodies, and anti-ds DNA antibodies were negative.
Implications:
- These cases highlight the importance of considering sarcoidosis in patients with unexplained sicca syndrome and extrapulmonary involvement.
- Glucocorticosteroid therapy demonstrated efficacy in managing extrapulmonary sarcoidosis symptoms.
- Further research into the immunopathogenesis of sarcoidosis presenting with sicca syndrome is warranted.