1. bookVolume 59 (2021): Issue 3 (September 2021)
Journal Details
License
Format
Journal
First Published
30 Mar 2015
Publication timeframe
4 times per year
Languages
English
access type Open Access

Pure extra-thoracic sarcoidosis: about 24 cases

Published Online: 26 Aug 2021
Page range: 312 - 317
Received: 07 Feb 2021
Journal Details
License
Format
Journal
First Published
30 Mar 2015
Publication timeframe
4 times per year
Languages
English
Abstract

Introduction. Pure extra-thoracic sarcoidosis is rare and poorly characterized in the literature. Herein we present features of pure extra-thoracic sarcoidosis, in comparison with sarcoidosis with intra-thoracic involvement, in a population from the south of Tunisia.

Methods. Retrospective, descriptive and comparative study of the patients presenting systemic sarcoidosis and attending the Department of Internal Medicine at the Hedi Chaker University Hospital, Sfax, Tunisia, for 21 years, from January 1996 to December 2016.

Results. Twenty-four patients presented pure extra-thoracic sarcoidosis (30% of the cases of systemic sarcoidosis). There was a female predominance (79%). The mean age was 50 years. The main features were polyadenopathies (10 patients: 41.7%), cutaneous involvement (10 patients: 41.6%), inflammatory polyarthralgia (8 cases: 33.3%), general symptoms (6 patients: 25%), uveitis (6 cases: 25%), cholestasis (3 cases) and kidney involvement (2 cases). Lymphopenia, hypercalcemia, and hypercalciuria were observed each one in 12.5% of the cases, and high angiotensin converting enzyme (ACE) level in 38.5% of the cases. The histological proof was required in all the patients. Statistically significant associations identified were mainly hepatic involvement with general symptoms (p=0.035), peripheral lymphadenopathies (p=0.035) and kidney damage (p=0.022), and cutaneous involvement with articular manifestations (p=0.032). Systemic corticosteroids were used in half of the cases, with a good outcome in 21 cases, and recurrence in two cases. One patient was lost to follow up. The comparison to the form with mediastino-pulmonary involvement showed less lymphadenopathies (p=0.001), less lymphopenia (p=0.006), and less frequent use of corticosteroids (p=0.044).

Conclusion. Our series was characterized by the frequency of the pure extra-thoracic form and the diversity of the systemic manifestations.

Keywords

1. SPAGNOLO P. Sarcoidosis: a Critical Review of History and Milestones. Clin Rev Allergy Immunol. 2015;49(1):1–5. Search in Google Scholar

2. FINGERLIN TE., HAMZEH N., MAIER LA. Genetics of Sarcoidosis. Clin Chest Med. 2015;36(4):569–584. Search in Google Scholar

3. SPAGNOLO P., ROSSI G., TRISOLINI R., SVERZELLATI N., BAUGHMAN RP., WELLS AU. Pulmonary sarcoidosis. The Lancet Respir Med. 2018;6(5):389–402. Search in Google Scholar

4. CHEN ES., MOLLER DR. Etiologies of Sarcoidosis. Clin Rev Allergy Immunol. 2015;49(1):6–18. Search in Google Scholar

5. BRITO-ZERON P., PEREZ-ALVAREZ R., PALLARES L., RETAMOZO S., BAUGHMAN RP., RAMOS-CASALSON M. Sarcoidosis: an update on current pharmacotherapy options and future directions. Expert Opinion on Pharmacotherapy. 2016;17(18):2431–2448. Search in Google Scholar

6. IPPERSIEL V., POCHET JM., DONCKIER J. Granulomatose médullaire: une présentation rare de sarcoïdose?. Louv Med. 2009;128(2):79–84. Search in Google Scholar

7. MOUTHON L., HANSLIK T., VILLARD J-F. Sarcoïdose. In: Médecine interne ECN, MED-LINE Editions, Paris, 2018. p. 261–77. Search in Google Scholar

8. VALEYRE D., DUPERRON F. Sarcoïdose: Dépistage et gestion des atteintes extra-pulmonaires. Rev Mal Respir. 2006;23(6):757–758. Search in Google Scholar

9. BART P., PRUVOT E., PRIOR J., WAEBER G. Sarcoïdose extrapulmonaire : entité méconnue ? Rev Med Suisse. 2010;6:2056–2060. Search in Google Scholar

10. JUDSON MA. Extrapulmonary Sarcoidosis. Semin Respir Crit Care Med. 2007;28:83–101. Search in Google Scholar

11. JUDSON MA., BAUGHMAN RP. How many organs need to be involved to diagnose sarcoidosis? An unanswered question that, hopefully, will become irrelevant. Sarcoidosis Vasc Diffus Lung Dis. 2014;31(1):19–27. Search in Google Scholar

12. BAUGHMAN RP., TEIRSTEIN AS., JUDSON MA., ROSSMAN MD., YEAGER JR H., BRESNITZ EA., et al. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med. 2001;164:1885–1889. Search in Google Scholar

13. ELLOUMI H., MARZOUK S., TAHRI N., BAHLOUL Z., AZOUZ MM. Sarcoïdose et atteinte hépatique: Étude de 25 cas. Rev Med Interne. 2012;33(11):607–614. Search in Google Scholar

14. JUDSON MA. The Clinical Features of Sarcoidosis: A Comprehensive Review. Clin Rev Allergy Immunol. 2015;49(1):63–78. Search in Google Scholar

15. VALEYRE D., JENY F., NUNES H. Current Medical Therapy for Sarcoidosis. Semin Respir Crit Care Med. 2017;38:523–531. Search in Google Scholar

16. HUNNINGHAKE GW. Statement on Sarcoidosis. Am J Respir Crit Care Med. 1999;160(3):736–755. Search in Google Scholar

17. VALEYRE D., PRASSE A., NUNES H., UZUNHAN Y., BRILLET P., MULLER-QUERNHEIM J. Sarcoidosis. Seminar. 2013;383(9923):1155–1167. Search in Google Scholar

18. ROCHAT TS., JANSSENS J-P., SOCCAL PM., ADLER D. Mise au point sur le traitement de la sarcoïdose. Rev Med Suisse. 2016;12:1966–1971. Search in Google Scholar

19. PRASSE A., KATIC C., GERMANN M., BUCHWALD A., ZISSEL G., MULLER-QUERNHEIM J. Phenotyping sarcoidosis from a pulmonary perspective. Am J Respir Crit Care Med. 2008;177(3):330–336. Search in Google Scholar

20. CHAPPEL A., CHEUNG W., HUTCHINGS H. Sarcoidosis: a long-term follow up study. Sarcoidosis Vasc Diffus Lung Dis. 2001;17(2):167–173. Search in Google Scholar

21. MORIMOTO T., AZUMA A., ABE S., USUKI J., KUDOH S., SUGISAKI K. et al. Epidemiology of sarcoidosis in Japan. Eur Respir J. 2008;31(2):372–379. Search in Google Scholar

22. OKUMUS G., MUSELLIM B., CETINKAYA E., TURKER H., UZASLAN E., YENTURK E., et al. Extrapulmonary involvement in patients with sarcoidosis in Turkey. Respirology. 2011;16(3):446–50. Search in Google Scholar

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