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Relapsing Polychondritis

Jonas F Ludvigsson, Ronald van Vollenhoven
No abstract text is available yet for this article.
2016: Clinical Epidemiology
Geetha Navuduri
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Chan Hong Jeon
Relapsing polychondritis (RP) is an autoimmune disorder characterized by inflammation in cartilaginous structures including the ears, noses, peripheral joints, and tracheobronchial tree. It rarely involves the central nervous system (CNS) but diagnosis of CNS complication of RP is challenging because it can present with varying clinical features. Herein we report 3 cases of relapsing polychondritis involving CNS with distinct manifestations and clinical courses. The first patient presented with rhombencephalitis resulting in brain edema and death...
November 2016: Journal of Korean Medical Science
Hakan Emmungil, Sibel Zehra Aydın
Relapsing polychondritis (RPC) is a unique and rarely observed autoimmune condition regarded as recurrent extensive chondritis of the auricular, nasal, and tracheal cartilages. Moreover, heart, main arteries, skin, and eyes may be involved. Several forms of clinical manifestations may be seen, and the pathogenesis still remains anonymous. A concomitant disease, particularly myelodysplasia or other systemic autoimmune disease can be detected in one-third of the patients with RPC. The treatment of RPC should be considered on personal basis and classified according to disease activity and severity...
December 2015: Eur J Rheumatol
AnneLiese Smylie, Neel Malhotra, Alain Brassard
Relapsing polychondritis, or RP, is a rare connective tissue disease characterized by relapsing-remitting destructive inflammation of the cartilaginous and other proteoglycan-rich structures in the body. Given the relatively low incidence of RP, a concise clinically relevant guide, focusing on the cutaneous manifestations of this serious disease, is lacking. In this review, we provide the dermatologist with an approach to diagnosing RP and a guide to its initial work-up, and management. We close with an overview of the currently available treatment modalities for RP...
October 1, 2016: American Journal of Clinical Dermatology
Aline Lauria Pires Abrão, Caroline Menezes Santana, Ana Cristina Barreto Bezerra, Rivadávio Fernandes Batista de Amorim, Mariana Branco da Silva, Licia Maria Henrique da Mota, Denise Pinheiro Falcão
Orofacial manifestations occur frequently in rheumatic diseases and usually represent early signs of disease or of its activity that are still neglected in clinical practice. Among the autoimmune rheumatic diseases with potential for oral manifestations, rheumatoid arthritis (RA), inflammatory myopathies (IM), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), relapsing polychondritis (RP) and Sjögren's syndrome (SS) can be cited. Signs and symptoms such as oral hyposalivation, xerostomia, temporomandibular joint disorders, lesions of the oral mucosa, periodontal disease, dysphagia, and dysphonia may be the first expression of these rheumatic diseases...
September 2016: Revista Brasileira de Reumatologia
Koichiro Wasano, Shuta Tomisato, Sayuri Yamamoto, Noriomi Suzuki, Taiji Kawasaki, Kaoru Ogawa
OBJECTIVE: To report the treatment efficacy of continual intratympanic steroid injection (ITSI) therapy in a patient with refractory sensorineural hearing loss accompanied by relapsing polychondritis. PATIENT: A 49-year-old female diagnosed with relapsing polychondritis at the age of 45 years and who had been treated with corticosteroids and immunosuppressants developed sensorineural hearing loss in the left ear. INTERVENTION: Her unilateral hearing loss did not recover despite receiving one cyclophosphamide pulse treatment, one methylprednisolone pulse treatment, and weekly leukapheresis...
August 20, 2016: Auris, Nasus, Larynx
Huijun Zhou, Minggang Su, Lin Li
BACKGROUND: Relapsing polychondritis (RP) is an uncommon autoimmune inflammatory disease that may affect cartilage throughout the body. CASE REPORT: We report on a case of fever of unknown origin in which 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) was performed to make a diagnosis of RP. CONCLUSION: Our case demonstrates that the use of 18F-FDG PET/CT is a useful diagnostic tool to accurately determine the extent of inflammation throughout the body which can be identified by an increased 18F-FDG uptake...
August 2016: Medicine (Baltimore)
V I Chernyak, Savel'ev A I, I V Men'shikova, A P Pogromov
Three clinical cases are described including two of relapsing polychondritis with lesions in the central and peripheral nervous system (one of long-standing aseptic lymphocytic meningitis and one of cranial neuropathy of 2, 5, 7, and 8 pairs) and the third case of the optic nerve lesion with amblyopia. The two former cases were successfully treated with high doses of corticosteroids, the third one with moderate doses of the same medications. The data from the current literature concerning variants of clinical manifestations, methods for diagnostics and treatment of neurologic manifestations of relapsing polychondritis are discussed...
2016: Klinicheskaia Meditsina
(no author information available yet)
No abstract text is available yet for this article.
July 2016: Journal of Computer Assisted Tomography
Anna Horváth, Nóra Páll, Katalin Molnár, Tamás Kováts, György Surján, Tamás Vicsek, Péter Pollner
OBJECTIVE: Relapsing polychondritis (RP) is a rare autoimmune inflammatory disease that attacks mainly cartilaginous structures or causes serious damage in proteoglycan-rich structures (the eyes, heart, blood vessels, inner ear). This study shows results regarding the epidemiology, progression, and associations of this highly variable disease by collecting all cases from a 124-million-person-year Central European nationwide cohort. METHODS: We used the Hungarian Health Care Database to identify all persons with possible RP infection...
2016: Clinical Epidemiology
Mari Yamasue, Shin-Ichi Nureki, Hiroyuki Matsumoto, Takamasa Kan, Takehiro Hashimoto, Ryoichi Ushijima, Yuko Usagawa, Jun-Ichi Kadota
Relapsing polychondritis (RP) is a rare systemic disorder characterized by recurrent, widespread chondritis of the auricular, nasal, and tracheal cartilages. IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease characterized by the infiltration of IgG4-bearing plasma cells into systemic organs. Although 25% to 35% of patients with RP have a concurrent autoimmune disease, coexistence of RP and IgG4-RD is rare. We herein report a case of RP complicated by IgG4-RD. A 63-year-old man developed recurrent bilateral ear pain and swelling, recurrent blurred and decreased vision, and migratory multiple joint pain, sequentially within one year...
2016: Tohoku Journal of Experimental Medicine
Jérémie Dion, Nathalie Costedoat-Chalumeau, Damien Sène, Judith Cohen-Bittan, Gaëlle Leroux, Charlotte Dion, Camille Francès, Jean-Charles Piette
OBJECTIVE: Relapsing polychondritis is a rare condition characterized by recurrent inflammation of cartilaginous tissues and systemic manifestations. Data on this disease remain scarce. METHODS: Retrospective study of patients with relapsing polychondritis seen between 2000 and 2012 in a single center, to describe patient characteristics and disease evolution, identify prognostic factors and define different clinical phenotypes. RESULTS: We included 142 patients with chondritis types (86 women [61%]; mean age at first symptoms 43...
June 22, 2016: Arthritis & Rheumatology
Sanae Shimamura, Naoto Yokogawa, Kengo Murata, Tetsuo Yamaguchi, Keisuke Uchida, Yoshinobu Eishi
Sarcoidosis is a systemic granulomatous disease that can affect any organ including the nose. Nasal crusting and congestion are common nasal symptoms of sarcoidosis, whereas cases of saddle-nose deformity are rarely reported. We describe here a case of sarcoidosis that presented with saddle nose resembling relapsing polychondritis. Since sarcoidosis shares clinical features with relapsing polychondritis, the differential diagnosis of saddle nose can be challenging without a clear pathology.
June 20, 2016: Modern Rheumatology
Yuka Kaneko, Noriaki Nakai, Takashi Kida, Yutaka Kawahito, Norito Katoh
Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome are disease that fulfilled criteria for diagnosis of Behcet's disease (BD) and relapsing polychondritis (RP). We report a 22-year-old Japanese woman presented with MAGIC syndrome and we described the clinicopathological characteristics of MAGIC syndrome based on a review of published cases from July 1985 to December 2015. In our case, the patient with oral aphthae, erythema nodosum, acne-like eruptions, uveitis, and polyarthritis fulfilled criteria for diagnosis of incomplete form of BD...
May 2016: Indian Journal of Dermatology
Firat Selvi, Justin Messina, William C Faquin, David A Keith
Relapsing polychondritis (RP) is a rare, multisystem autoimmune disease characterized by inflammation, structural damage, and impaired function of cartilaginous tissues throughout the body. In the craniofacial region, this rare disease has been reported to affect structures of the ear and nose; however, reports of temporomandibular joint (TMJ) involvement are scarce. A second uncommon disorder of cartilage is synovial chondromatosis (SC), a progressive and proliferative disorder of the synovial membrane associated with the formation of variably sized cartilaginous and calcified loose bodies, often causing dysfunction of the joints and enlargement of the joint capsule...
May 18, 2016: Journal of Oral and Maxillofacial Surgery
Michelle A Nguyen, Sahand Rahnama-Moghadam, Robert T Gilson
Relapsing polychondritis (RP) is a rare rheumatologic disorder in which recurrent episodes of inflammation result in destruction of cartilage of the ears and nose. The joints, eyes, audio-vestibular system, heart valves, respiratory tract, kidneys, and skin can also be involved. Skin involvement is most frequently linked to concomitant myelodysplastic syndrome and has rarely been associated with systemic lupus erythematosus. A 47-year-old woman presented with violaceous, indurated, tender plaques on the bilateral cartilaginous ears with sparing of the lobes, consistent with RP...
2016: Dermatology Online Journal
Lijun Liu, Shengyun Liu, Wenjuan Guan, Lei Zhang
Relapsing polychondritis (RP) rarely affected the central nervous system (CNS). If the CNS is involved, it can result in psychiatric manifestations. Patients with RP always respond well to glucocorticoids and immunosuppressants. If the therapies fail, biologics can be given, such as tocilizumab, which is a humanized monoclonal antibody against the interleukin-6 receptor (IL-6R). Until now, there have been no randomized clinical trials to test the safety and efficacy of biologics, no reports of RP with psychiatric disorders as initial symptoms, and no reports of tocilizumab used for psychiatric symptoms due to RP...
August 2016: Rheumatology International
Chikashi Terao, Hajime Yoshifuji, Yoshihisa Yamano, Hiroto Kojima, Kimiko Yurugi, Yasuo Miura, Taira Maekawa, Hiroshi Handa, Koichiro Ohmura, Hiroh Saji, Tsuneyo Mimori, Fumihiko Matsuda
OBJECTIVE: To uncover the genetic background of relapsing polychondritis (RPC), a rare autoimmune disease with unknown mechanisms characterized by systemic inflammation of the cartilage, to deepen our understanding of the pathophysiology of RPC and show its distinct genetic characteristics from other rheumatic diseases. METHODS: A total of 102 patients with RPC and 1000 healthy subjects were recruited for a two-staged genetic association study and genotyped for six HLA classical loci...
September 2016: Rheumatology
Mateus Mistieri Simabukuro, Leandro Tavares Lucato, Samuel Katsuyuki Shinjo, Wellington Lima Flores, Luiz Henrique Martins Castro
No abstract text is available yet for this article.
May 17, 2016: Neurology
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