Association of sarcoidosis and immune thrombocytopenia: presentation and outcome in a series of 20 patients
| Autor: | Thomas Papo, Louis Affo, Matthieu Mahévas, Bertrand Godeau, Marc Michel, Anne Sophie Morin, Olivier Lidove, Guillaume Le Guenno, Dominique Valeyre, Laurent Chiche, Yurdagul Uzunhan, Geneviève Dion, David Boutboul, Véronique Péronne, Nicolas Schleinitz, Jean-Pierre Ducroix, Yves Pacheco, Medhi Khellaf |
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| Rok vydání: | 2011 |
| Předmět: |
Adult
medicine.medical_specialty Adolescent Sarcoidosis Comorbidity Gastroenterology Risk Assessment Severity of Illness Index Cohort Studies Young Adult Pharmacotherapy Age Distribution Prednisone hemic and lymphatic diseases Internal medicine Severity of illness Medicine Humans Young adult Sex Distribution Aged Retrospective Studies Aged 80 and over Purpura Thrombocytopenic Idiopathic business.industry Immunoglobulins Intravenous Retrospective cohort study General Medicine Middle Aged medicine.disease Surgery Treatment Outcome Concomitant Drug Therapy Combination Female business medicine.drug Follow-Up Studies |
| Zdroj: | Medicine. 90(4) |
| ISSN: | 1536-5964 |
| Popis: | The association of sarcoidosis and immune thrombocytopenia (ITP) has rarely been investigated. The aim of the current retrospective study was to investigate the clinical and biological phenotypes and outcome of this association in a large series of recent patients. Twenty patients (50% men) were included. Median age at sarcoidosis and ITP diagnosis was 36 (range, 10-83 yr) and 38 (range, 21-83 yr) years, respectively. In 11 of 20 (55%) patients, sarcoidosis onset preceded ITP (median interval, 48 mo; range, 6-216 mo). In 5 of 20 (25%) patients, the 2 conditions occurred concomitantly. In 4 of 20 (20%) patients, ITP onset preceded sarcoidosis (median interval, 68 mo; range, 15-153 mo). In 4 cases, sarcoidosis and ITP were not concomitant, since 1 condition was cured before the other was declared. In 12 of 20 (60%) patients there was a simultaneous onset or relapse of both ITP and sarcoidosis. Sarcoidosis phenotype was characterized by an acute onset in 40% of patients. The visceral involvement included thoracic sites in 19 of 20 (95%) patients and extrathoracic sites in 16 of 20 (80%) patients. At ITP onset, median platelet count was 11 × 10/L (range, 3-90); 17 (85%) patients had a platelet count30 × 10/L. Seven (35%) patients had a bleeding score8 without visceral bleeding.Nineteen of the 20 (95%) patients were treated specifically for ITP. After the first-line therapy (prednisone at 1 mg/kg per day for at least 3 consecutive weeks in all patients; with IVIg in addition for 10 patients with severe bleeding score), 12 of 19 (63%) patients achieved a complete response, 6 (31.5%) had a partial response, and only 1 patient failed to respond. At the end of ITP follow-up (median, 70 mo; range, 12-142 mo), 18 (90%) patients achieved a complete response, 1 achieved a partial response, and 1 had no response. After a median follow-up of 105 months, 13 of 20 (65%) patients had persistent sarcoidosis requiring prolonged therapy, and thus sarcoidosis represented the main long-term concern. Main conclusions were 1) ITP presentation was usually severe, but response to treatment was favorable in almost all cases, with no death and no severe bleeding, in contrast with older reports, 2) sarcoidosis was remarkable for the high proportion of cases with an acute onset, a chronic course, and the need for prolonged prednisone therapy, 3) sarcoidosis and ITP onset and evolution were not always synchronous. |
| Databáze: | OpenAIRE |
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