title: Association of sarcoidosis and immune thrombocytopenia: presentation and outcome in a series of 20 patients.
abstract: 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 count <30 × 10/L. Seven (35%) patients had a bleeding score >8 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.
authors.affiliation: From Department of Internal Medicine-Centre de référence des cytopénies auto-immunes de l'adulte (M. Mahévas, MK, M. Michel, BG), Henri Mondor Hospital, Assistance Publique Hôpitaux de Paris, Paris XII University, Créteil; Groupe Sarcoidose Francophone (M. Mahévas, YU, GD, JPD, YP, BG, DV), SPLF, Maison du poumon, Paris; Department of Internal Medicine (LC, NS), Hôpital de la Conception, Marseille; Université Paris 13, EA2363 and Avicenne Hospital, Department of Pneumology (YU, GD, DV), Assistance Publique Hôpitaux de Paris, Bobigny; Department of Internal Medicine (ASM), Jean-Verdier Hospital, Bondy; Departement of Internal Medicine (GLG), Clermont Ferrand Hospital, Clermont Ferrand; Department of Internal Medicine (VP), François Quesnay Hospital, Mantes la Jolie; Department of Internal Medicine(LA), Louis Mourier Hospital, Assistance Publique Hôpitaux de Paris, Colombes; Department of Internal Medicine (OL, TP), Bichat Hospital, Assistance Publique Hôpitaux de Paris, Claude Bernard, Paris; EA 3963, Centre Hayem, Saint-Louis Hospital (DB), Assistance Publique Hôpitaux de Paris, Paris; Department of Internal Medicine (JPD), Amiens Nord Hospital, Jules Verne University, Amiens; Department of Pneumology (YP), Lyon Hospital, Université Lyon sud, Lyon; France.
authors.forename: Matthieu
authors.initials: M
authors.lastname: Mahévas
authors.forename: Laurent
authors.initials: L
authors.lastname: Chiche
authors.forename: Yurdagul
authors.initials: Y
authors.lastname: Uzunhan
authors.forename: Medhi
authors.initials: M
authors.lastname: Khellaf
authors.forename: Anne Sophie
authors.initials: AS
authors.lastname: Morin
authors.forename: Guillaume
authors.initials: G
authors.lastname: Le Guenno
authors.forename: Véronique
authors.initials: V
authors.lastname: Péronne
authors.forename: Louis
authors.initials: L
authors.lastname: Affo
authors.forename: Olivier
authors.initials: O
authors.lastname: Lidove
authors.forename: David
authors.initials: D
authors.lastname: Boutboul
authors.forename: Genevieve
authors.initials: G
authors.lastname: Dion
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Ducroix
authors.forename: Thomas
authors.initials: T
authors.lastname: Papo
authors.forename: Yves
authors.initials: Y
authors.lastname: Pacheco
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
authors.forename: Marc
authors.initials: M
authors.lastname: Michel
authors.forename: Bertrand
authors.initials: B
authors.lastname: Godeau
authors.forename: Dominique
authors.initials: D
authors.lastname: Valeyre
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D015897:Comorbidity; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D012189:Retrospective Studies; D018570:Risk Assessment; D012507:Sarcoidosis / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology*; D012720:Severity of Illness Index; D017678:Sex Distribution; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D016756:Immunoglobulins, Intravenous; D011241:Prednisone