Salam Abbara1, Sophie Georgin-Lavialle2, Katia Stankovic Stojanovic1, Claude Bachmeyer1, Patricia Senet3, David Buob4, Sylvain Audia5, Véronique Delcey6, Soraya Fellahi7, Jean-Philippe Bastard7, Fawaz Awad8, Marie Legendre9, Serge Amselem10, Gilles Grateau11. 1. Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, Assistance publique-Hôpitaux de Paris (AP-HP), université Paris 6, Pierre-et-Marie-Curie (UPMC), 20, rue de la Chine, 75020 Paris, France. 2. Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, Assistance publique-Hôpitaux de Paris (AP-HP), université Paris 6, Pierre-et-Marie-Curie (UPMC), 20, rue de la Chine, 75020 Paris, France; Inserm UMRS_933, hôpital Trousseau, AP-HP université Pierre-et Marie-Curie (UPMC)-Paris 6, Paris, France. Electronic address: sophie.georgin-lavialle@aphp.fr. 3. Service de dermatologie, hôpital Tenon, AP-HP, UPMC, 20, rue de la Chine, 75020 Paris, France. 4. Laboratoire d'anatomie pathologique, hôpital Tenon, AP-HP, UPMC, 20, rue de la Chine, 75020 Paris, France. 5. Service de médecine interne et immunologie clinique, Bocage Central, 14, rue Paul-Gaffarel, 21000 Dijon, France. 6. Service de médecine interne, hôpital Lariboisière, AP-HP, 2, rue Ambroise-Paré, 75010 Paris, France. 7. Laboratoire de biochimie, hôpital Tenon, 20 rue de la Chine, AP-HP, UPMC, 75020 Paris, France. 8. Inserm UMRS_933, hôpital Trousseau, AP-HP université Pierre-et Marie-Curie (UPMC)-Paris 6, Paris, France; Laboratoire de génétique, hôpital Trousseau, AP-HP, UPMC, 26, rue du Dr-Arnold-Netter, 75012 Paris, France. 9. Laboratoire de génétique, hôpital Trousseau, AP-HP, UPMC, 26, rue du Dr-Arnold-Netter, 75012 Paris, France. 10. Inserm UMRS_933, hôpital Trousseau, AP-HP université Pierre-et Marie-Curie (UPMC)-Paris 6, Paris, France; Service de dermatologie, hôpital Tenon, AP-HP, UPMC, 20, rue de la Chine, 75020 Paris, France; Laboratoire de génétique, hôpital Trousseau, AP-HP, UPMC, 26, rue du Dr-Arnold-Netter, 75012 Paris, France. 11. Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, Assistance publique-Hôpitaux de Paris (AP-HP), université Paris 6, Pierre-et-Marie-Curie (UPMC), 20, rue de la Chine, 75020 Paris, France; Inserm UMRS_933, hôpital Trousseau, AP-HP université Pierre-et Marie-Curie (UPMC)-Paris 6, Paris, France.
Abstract
OBJECTIVES: Familial mediterranean fever (FMF) is the most common monogenic autoinflammatory disease. Hidradenitis suppurativa (HS) is an inflammatory cutaneous disease. Those diseases can occur simultaneously among the same individual. Our objective was to describe the features of patients displaying both FMF and HS. METHODS: We screened the French adult FMF reference center for FMF patients with HS. RESULTS: Six patients out of 151 (4%) with a median age of 36 years old were concerned. Among them, FMF was symptomatic at a median age of 11.5years old and colchicine was introduced at a median age of 20.5years old. HS was diagnosed at a median age of 31.5years old. An elderly patient displayed AA amyloidosis in the outcome of FMF, with a late diagnosis of HS, with response to anakinra. There was no temporal relation between FMF and HS attacks. Some patients had a persistent inflammatory syndrome under treatment. CONCLUSION: FMF and HS are both inflammatory diseases involving young patients, with HS possibly being an autoinflammatory disease. Although their association seems to be fortuitous, both can induce an important inflammation state that could lead to AA amyloidosis and require a close monitoring of clinical signs and acute-phase reactants. Anakinra was successful in treating the only patient with both HS, FMF and amyloidosis.
OBJECTIVES:Familial mediterranean fever (FMF) is the most common monogenic autoinflammatory disease. Hidradenitis suppurativa (HS) is an inflammatory cutaneous disease. Those diseases can occur simultaneously among the same individual. Our objective was to describe the features of patients displaying both FMF and HS. METHODS: We screened the French adult FMF reference center for FMFpatients with HS. RESULTS: Six patients out of 151 (4%) with a median age of 36 years old were concerned. Among them, FMF was symptomatic at a median age of 11.5years old and colchicine was introduced at a median age of 20.5years old. HS was diagnosed at a median age of 31.5years old. An elderly patient displayed AA amyloidosis in the outcome of FMF, with a late diagnosis of HS, with response to anakinra. There was no temporal relation between FMF and HS attacks. Some patients had a persistent inflammatory syndrome under treatment. CONCLUSION:FMF and HS are both inflammatory diseases involving young patients, with HS possibly being an autoinflammatory disease. Although their association seems to be fortuitous, both can induce an important inflammation state that could lead to AA amyloidosis and require a close monitoring of clinical signs and acute-phase reactants. Anakinra was successful in treating the only patient with both HS, FMF and amyloidosis.