A Case of Polychondritis in a Patient with Behçet's Disease

Abstract

Polychondritis is an inflammatory disorder that affects various catilagenous structures, and the clinical features include auricular, nasal and respiratory tract chondritis. It also involves the eyes, audiovestibular apparatus, joints and vascular structures. Polychondritis can be associated with several rheumatologic diseases such as systemic lupus erythematosus, rheumatoid arthritis and systemic vasculitis. However, polychondritis is a rare complication of Behçet's disease (BD) and only ten cases with combined BD and polychondritis have been reported on around the world. In this report, we describe a 40-year-old Korean man with BD who suffered from polychondritis that manifested as bilateral auricular chondritis, conjunctivitis and arthritis.

Optic Neuropathy Associated With Periostitis in Relapsing Polychondritis

“…Moreover, pachymeningitis is reported in conditions similar to RPC, including rheumatoid arthritis (37)(38)(39), Wegener granulomatosis (WG) (40,41), and p-ANCA-positive microscopic polyangiitis (42)(43)(44)(45)(46). Indeed, WG (47,48), ankylosing spondylitis (49), lupus (50), and Behc xet disease (51) have been reported to manifest auricular chondritis.…”

Ileocecal ulcers accompanied by relapsing polychondritis: a case report

“…MAGIC syndrome is defined as an overlap syndrome that includes features characteristic of both BD and RP. Although a few authors suggest that MAGIC syndrome is not a disease entity but merely the association of BD with polychondritis (Kotter et al 2006 ; Kotter 2006 ), many authors support the concept of this syndrome (Imai et al 1997 ; Orme et al 1990 ; Kim et al 2005 ; Caceres et al 2006 ; Nanke et al 2006 ; Hidalgo-Tenorio et al 2008 ; Mekinian et al 2009 ; Geissal and Wernick 2010 ; Gertner 2004 ). They suggest that the similarities in the clinical manifestations and pathological findings in BD and RP lead a common pathogenetic pathway, so that there is a close relationship between both diseases (Orme et al 1990 ; Kim et al 2005 ; Hidalgo-Tenorio et al 2008 ; Mekinian et al 2009 ; Gertner 2004 ).…”

“…However, both mucosal symptoms (oral aphthae or genital ulcers) and cartilage inflammation are thought to be necessary for diagnosis according to the first descriptions by Firestein et al ( 1985 ). It is a very uncommon disorder, and until now, only 24 cases have been reported (Firestein et al 1985 ; Imai et al 1997 ; Kotter et al 2006 ; Minami et al 2009 ; Orme et al 1990 ; Kim et al 2005 ; Caceres et al 2006 ; Nanke et al 2006 ; Hidalgo-Tenorio et al 2008 ; Mekinian et al 2009 ; Geissal and Wernick 2010 ; Gertner 2004 ; Le Thi et al 1993 ; Fernandez-Monras et al 1997 ; Ng et al 2007 ). All of the 24 cases had both mucosal symptoms and cartilage inflammation, and met the Firestein’s definition.…”

Manifestasi Behçet Disease yang Parah dan Komplikasi Perawatannya dalam Rongga Mulut

“…Behçet's Disease (BD) merupakan suatu peradangan multisistem kronis yang ditandai oleh ulserasi yang hilang timbul pada rongga mulut, alat kelamin, mata dan umumnya melibatkan sendi, kulit, sistem saraf pusat dan saluran pencernaan. [1][2][3][4][5][6] Selain ulserasi rongga mulut, manifestasi klinis di organ lain sangat bervariasi pada tiap populasi yang berbeda. Hal ini berkaitan dengan adanya pengaruh genetik terhadap timbulnya serta perkembangan penyakit.…”

“…1 Manifestasi BD dapat terjadi pula pada berbagai sistem organ di antaranya trombosis, gangguan neurologis (keterlibatan sistem saraf pusat parenkim dan hipertensi intrakranial), gangguan kardiovaskular (aneurisma arteri dan vaskulitis pembuluh darah kecil), ulserasi intestinal serta polikondritis. 1,4,9 Penyakit ini sebagai suatu kesatuan penyakit, pertama kali digambarkan oleh dokter ahli penyakit kulit yang berasal dari Turki bernama Hulusi Behçet pada tahun 1937 yang menyebutnya sebagai "ulser aftosa rekuren, ulser genital dan uveitis-hipopion". 3,5,10 Prevalensi BD di dunia sangat bervariasi tergantung pada etnis dan daerah geografis tertentu.…”