Manifestations mimicking relapsing polychondritis in a patient with microscopic polyangiitis
Nihon Rinsho Meneki Gakkai Kaishi. 2005 Apr;28(2):104-8.
[Article in Japanese]
The Second Department of Internal Medicine, Saitama Medical Center.
Ogawa H, Nishi E, Kameda H, Amano K, Takeuchi T.
Microscopic polyangiitis (MPA) is a systemic disorder characterized by inflammation of small vessels mainly affecting the kidneys and lungs. We describe a 72-year-old woman who developed multiple cartilage involvements as well as major manifestations of MPA. The left ear biopsy demonstrated cartilaginous inflammation and small vessel vasculitis. She also had conjunctivitis, hearing impairment, interstitial lung disease, glomerulonephritis with vasculitis and mononeuritis multiplex. Serological examinations revealed a positive antineutrophil cytoplasmic antibody (PR-3 ANCA). Cyclophosphamide and oral corticosteroid therapy was instituted and remission achieved. Due to lacks of nasal and bronchial involvements, as well as the evidence of auricular vasculitis, we concluded that her findings mimicking relapsing polychondritis developed as systemic manifestations of MPA.
Relapsing polychondritis - a case report and review of the literature.
Laryngorhinootologie. 2005 May;84(5):352-6
[Article in German]
Tesche S, Wenzel S, Sagowski C.
Klinik und Poliklinik fur Hals-, Nasen-und Ohrenheilkunde Universitatsklinikum Hamburg-Eppendorf (Direktor: Univ.-Prof. Dr. med. U. Koch).
PATIENT: A case of a 54-year-old woman with a three month history of recurrent bilateral chondritis of the auricles, cochlear and vestibular inner ear damage and conjunctivitis is described. The diagnosis was based only on clinical criteria (auricular chondritis, conjunctivitis, inner ear damage). Antinuclear antibodies, ANCA and HLA-DR 4 antigen were negative. The only laboratory abnormality was an elevated erythrocyte sedimentation rate. The patient has been treated successfully with Methotrexate 20 mg 1 x /week and Prednisone 15 mg/die for 4 month now. DISCUSSION: The relapsing polychondritis (RP) is a rare, multisystemic and potentially fatal disease. The pathogenesis and optimal therapeutic approach is poorly understood. The disease is characterized by episodic inflammation of cartilage such as auricular, nasal and laryngotracheal. Many other proteoglycan-rich structures like inner ear, eye, kidney and blood vessels, may be involved as well. RP has an equal sex prevalence. The majority of cases appear between 40 and 60 years. Auricular inflammation is the most common feature. Effectiveness of non-steroidal anti-inflammatory drugs, dapsone, immunosuppressive drugs and prednisone has been described. The overall survival rates were 74 % at 5 years and 55 % at 10 in one 1986 series. CONCLUSION: The most common clinical presentation of RP regularly involves ENT-structures. Therefore ENT-specialists should be familiar with this disease. A close interdisciplinary cooperation is essential for therapy and follow-up, because pulmonary and cardiac involvement are limiting prognostic factors.
Relapsing polychondritis with aortitis without valvular involvement.
J Rheumatol. 2005 May;32(5):954-6
Rho YH, Choi SJ, Choi YS, Lee YH, Ji JD, Song GG.
Division of Rheumatology, Korea University Guro Hospital, Seoul, South Korea.
Relapsing polychondritis is a multisystemic disease of unknown etiology that mainly involves the cartilaginous portions of the ear, nose, and trachea. Occasionally, there is involvement of the cardiovascular system, which usually results in severe morbidity and mortality. The most common manifestation of cardiovascular involvement is aortic root dilation resulting in aortic regurgitation. We describe the first case in Korea, a 51-year-old woman with relapsing polychondritis with aortitis, but without aortic valve involvement. She presented only with complaints of abdominal pain, and was successfully treated with corticosteroids and methotrexate.
Brain magnetic resonance imaging and single photon emission computerized tomography findings in a case of relapsing polychondritis showing cognitive impairment and personality changes.
Prog Neuropsychopharmacol Biol Psychiatry. 2005 Feb;29(2):347-9 Epub 2005 Jan 8.
Ota M, Mizukami K, Hayashi T, Sumida T, Asada T.
Department of Psychiatry, Institute of Clinical Medicine, University of Tsukuba, Ibaraki 305-8575, Japan.
A 57-year-old female with relapsing polychondritis (RP) showing CNS complications is described with reference to the MRI and SPECT findings. At the age of 56, the patient was diagnosed with RP based on her symptoms, which included bilateral auricular chondritis, nonerosive seronegative inflammatory polyarthritis, and ocular inflammation, as well as vestibular dysfunction. During the course of the disease, she suffered from aseptic meningitis accompanied by delirium, which was resolved by treatment with cyclophosphamide, in addition to prednisolone concurrent with the improvement of her physical condition. After the meningitis improved, she developed a number of cognitive impairments, including time disorientation, agraphia, acalculia, constitutional apraxia, and personality changes. Simultaneously, we observed, via SPECT, a decrease in cerebral blood flow (CBF) in several regions, including the bilateral frontal, lateral temporal, and parietal regions. This is the first study to report on the CNS manifestations of RP with reference to MRI and SPECT findings.
Obstructive sleep apnoea/hypopnoea as the initial presentation of relapsing polychondritis.
Int J Clin Pract. 2004 Jan;58(1):97-9.
Riha RL, Douglas NJ.
Department of Medicine, Royal Infirmary Edinburgh, Edinburgh, UK.
Relapsing polychondritis (RP) is a relatively rare rheumatological condition, which can affect the upper airway and prove potentially life threatening. We report the first case of RP presenting initially as the obstructive sleep apnoea/hypopnoea syndrome, discuss management and review the extant literature.
Relapsing polychondritis: reversible airway obstruction is not always asthma.
Mayo Clin Proc. 2004 Mar;79(3):407-9.
Segel MJ, Godfrey S, Berkman N.
Institute of Pulmonology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation of cartilaginous and other proteoglycan-rich tissues. Respiratory tract involvement is a common cause of morbidity and mortality in RP. We describe a patient whose clinical features at onset of disease were typical of asthma. Later, the patient developed symptoms and signs characteristic of RP. Tracheobronchomalacia necessitated airway support by stenting. The possibility that airway obstruction in the initial stages of RP is due to airway inflammation and that early, aggressive immunosuppressive treatment of RP may delay or prevent irreversible cartilaginous destruction and airway collapse are discussed.
Purulent chondritis of the laryngeal framework cartilages.
Ann Otol Rhinol Laryngol. 2005 Mar;114(3):219-22.
Eliashar R, Gross M, Goldfarb A, Sichel JY.
Department of Otolaryngology-Head and Neck Surgery, The Hebrew University School of Medicine, Hadassah Medical Center, Jerusalem, Israel.
This manuscript reports on our experience with purulent chondritis of the laryngeal cartilages (PCLC), an entity that has not yet been described. Three patients had a diagnosis of PCLC. The probable causes were relapsing polychondritis, a previous prolonged intubation, and an idiopathic cause. The patients suffered from hoarseness and inspiratory stridor for 1 to 3 months before diagnosis. None complained of pain in the neck. Laryngoscopy showed supraglottic edema. A computed tomography scan revealed abscess formation between the intact inner and outer perichondria of the thyroid cartilage. The treatment included rigid endoscopy, external incision and drainage, and prolonged medical therapy. The culture results were Staphylococcus aureus in the first 2 cases and Aspergillus fumigatus in the third. The second patient (in whom the cricoid cartilage was also affected) required emergency tracheotomy. The other 2 patients did not require airway intervention. The rarity of PCLC and the relatively mild symptoms require a high index of suspicion for its diagnosis.
