phenylephrine-hydrochloride and Polychondritis--Relapsing

Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation and destruction of the cartilaginous structures. Tracheobronchial chondritis is a dreaded complication of RP. We wish to report a case of RP of the trachea and bronchi which was treated with nasal continuous positive airway pressure.

Topics: Fatal Outcome; Female; Humans; Masks; Middle Aged; Nose; Polychondritis, Relapsing; Positive-Pressure Respiration; Stents; Tracheal Diseases

Topics: Disease Management; Female; Humans; Immunosuppression Therapy; Immunosuppressive Agents; Middle Aged; Nose; Plastic Surgery Procedures; Polychondritis, Relapsing; Respiratory System

Topics: Abscess; Aged; Anti-Bacterial Agents; Antirheumatic Agents; Diagnosis, Differential; Ear Auricle; Humans; Lymphohistiocytosis, Hemophagocytic; Male; Methotrexate; Neck; Nose; Polychondritis, Relapsing; Prednisone; Propionibacterium acnes; Tomography, X-Ray Computed; Treatment Outcome

Topics: Adult; Ear; Glucocorticoids; Humans; Male; Nose; Polychondritis, Relapsing; Prednisone

We present the case of a 39-year-old man with a severe glottis stenosis. The saddle nose, images of laryngotracheal stenosis and the (FDG) positron-emission tomography/computed tomography lead to a final diagnosis of relapsing polychondritis. In the patient a coexistent myelodysplastic syndrome was diagnosed. Moreover, the elevated total IgG4 exceeding 135 ml/dl requested additional immunochemistry for detection of IgG4-bearing plasma cells in the biopsies. The patient underwent an allogenic stem cell transplantation and died on day 40 after the transplantation because of an acute steroid-resistent graft vs host.

Topics: Adult; Constriction, Pathologic; Glottis; Humans; Male; Nose; Polychondritis, Relapsing

Relapsing polychondritis (RP) is a rare disease which presents chondritis in multiple organs. Characteristic features include auricular chondritis, arthritis, nasal chondritis, ocular inflammation, respiratory tract involvement and audiovestibular damage. Fifty percent of cases of RP are associated with inner ear symptoms such as dizziness and hearing loss. We have recently encountered a case of RP in a 34-year-old man who had recurrent chondritis of both auricles and progressive bilateral profound sensorineural hearing loss; he had been treated many times with steroids, immunosuppressants, plasmapheresis treatments. We perfomed a successful cochlear implant surgery on the left ear of this patient. This raises the possibility of using cochlear implants in treating patients with immune-mediated inner ear disease as well as such RP patients.

Topics: Adult; Cochlear Implantation; Ear Auricle; Hearing Loss, Sensorineural; Humans; Male; Nose; Polychondritis, Relapsing; Treatment Outcome

Topics: Acute Disease; Adolescent; Humans; Male; Nose; Polychondritis, Relapsing; Radiography; Respiratory Insufficiency; Trachea

Relapsing polychondritis is a rare systemic disease. Clinical manifestations are variable and the condition is frequently associated with other diseases. Chondritis typically involves ears, nose, costal cartilages and upper airways. Other classical features include scleritis and episcleritis, mucocutaneous lesions, arthralgia and constitutional symptoms. Repeated inflammation of cartilaginous structures may lead to deformities of the ears, the nose and the airways. Tracheobronchomalacia and aortitis of the ascending portion resulting in aortic regurgitation or aneurysm are feared complications. Treatment is mainly based on systemic corticosteroids alone or in association with immunosuppressants. Several biological immunosuppressive agents are reported effective in refractory disease.

Topics: Adrenal Cortex Hormones; Aneurysm; Anti-Inflammatory Agents, Non-Steroidal; Aortic Valve Insufficiency; Aortitis; Diagnosis, Differential; Drug Therapy, Combination; Ear, External; Humans; Immunosuppressive Agents; Laryngeal Diseases; Nose; Polychondritis, Relapsing; Scleritis; Skin; Tracheal Diseases; Tracheobronchomalacia; Treatment Outcome

Topics: Adolescent; Anti-Inflammatory Agents; Antibodies, Monoclonal; Female; Follow-Up Studies; Humans; Infliximab; Nose; Nose Deformities, Acquired; Plastic Surgery Procedures; Polychondritis, Relapsing; Tumor Necrosis Factor-alpha

Relapsing polychondritis (RP) is a human autoimmune disease of unknown etiology in which cartilaginous sites are destroyed by cyclic inflammatory episodes beginning, most commonly, during the fourth or fifth decade of life. We have previously described collagen-induced polychondritis that closely mirrors RP occurring in young (6-8 weeks old) HLA-DQ6alphabeta 8alphabeta transgenic Abeta0 mice, following immunization with heterologous type II collagen (CII). We present evidence here that transgenic strains expressing the DQ6alpha8beta transgene develop spontaneous polychondritis (SP) at the mouse equivalent of human middle age (4.5-6 months and 40-50 years old, respectively) and display polyarthritis, auricular chondritis and nasal chondritis--three of the most common sites affected in RP. Auricular chondritis in SP, like RP but unlike CII-induced polychondritis, exhibited a relapsing/remitting phenotype, requiring several inflammatory cycles before the cartilage is destroyed. Elevated serum levels of total IgG corresponded with the onset of disease in SP, as in RP and CII-induced polychondritis. No CII-specific immune response was detected in SP, however--more closely mirroring RP, in which as few as 30% of RP patients have been reported to have CII-specific IgG. CII-induced polychondritis displays a strong CII-specific immune response. SP also demonstrated a strong female preponderance, as some workers have reported in RP but has not observed in CII-induced polychondritis. These characteristics of SP allow for the examination of the immunopathogenesis of polychondritis in the absence of an overwhelming CII-specific immune response and the strong adjuvant-induced immunostimulatory influence in CII-induced polychondritis. This spontaneous model of polychondritis provides a new and unique tool to investigate both the initiatory events as well as the immunopathogenic mechanisms occurring at cartilaginous sites during the cyclic inflammatory assaults of polychondritis.

Topics: Animals; Cartilage; Disease Models, Animal; Ear Cartilage; Female; HLA-DQ Antigens; Immunoglobulin G; Male; Mice; Mice, Transgenic; Nose; Phenotype; Polychondritis, Relapsing; Sex Factors; Transgenes

A 46-year-old female with relapsing polychondritis (RP) was scheduled for surgical repair of saddle-nose and peri-chondritic ear. RP is a rare systemic, inflammatory, and destructive disease of the cartilaginous structures leading to multiple functional disorders in the affected organs. Preoperatively, her chest radiogram of the trachea showed narrowing in diameters which altered about 2 mm with expiration and inspiration. Propofol administered intravenously was given as induction and anesthesia was maintained with nitrous oxide-oxygen-sevoflurane with spontaneous ventilation using laryngeal mask airway. The operation was over uneventfully. Anesthesiologic management requires careful preoperative evaluation of vital organ functions, in particular, respiration.

Topics: Anesthesia, Inhalation; Anesthetics, Inhalation; Anesthetics, Intravenous; Ear; Female; Humans; Methyl Ethers; Middle Aged; Nitrous Oxide; Nose; Oxygen; Polychondritis, Relapsing; Propofol; Sevoflurane

We report the case of a 46-year-old patient in whom ulcerative colitis had been diagnosed three years ago. He was admitted to the hospital for swelling of the nose. Clinical course and complementary exams led us to diagnose atrophic polychondritis. Twelve cases of such an association have been published so far.

Topics: Colitis, Ulcerative; Humans; Male; Middle Aged; Nose; Polychondritis, Relapsing; Prednisolone; Technetium

Topics: Adult; Beauty Culture; Cricoid Cartilage; Ear; Ear Cartilage; Female; Glottis; Humans; Immunosuppressive Agents; Inflammation; Methotrexate; Needles; Nose; Polychondritis, Relapsing; Prednisone; Treatment Outcome; Wounds, Penetrating

Relapsing polychondritis is a rare and little known inflammatory disease. The case of a 29-year-old woman who presented with a one-year history of saddle nose is discussed in this article. After waiting for one year, rhinoplasty was decided, with a good result at the 18th month. We prefer to use a calvarial bone graft for this disease and to only operate under stable and minor conditions.

Topics: Adult; Female; Humans; Nose; Plastic Surgery Procedures; Polychondritis, Relapsing

An auricular or nasal chondritis or a saddle nose deformity are the initial manifestation in half of cases of relapsing polychondritis; the other initial manifestations are various and less evocative; polyarthritis, laryngo-tracheal symptoms, episcleritis which delay the diagnosis. From 15 cases of relapsing polychondritis, the diagnosis time from the first symptom are studied; this one is long, about 3 years and 6 months (from 3 months to 17 years) in 13/15 of the cases even if the first manifestation is typical (external chondritis). In two cases only, the diagnosis was established after the first attack. This late of diagnosis had socioeconomical impact and exposed to severe complications like tracheal chondritis.

Topics: Adult; Aged; Cartilage Diseases; Diagnostic Errors; Ear Cartilage; Female; Humans; Laryngeal Diseases; Male; Middle Aged; Nose; Polychondritis, Relapsing; Retrospective Studies; Time Factors; Trachea

Relapsing poly(peri)chondritis (RP) is a connective tissue disorder characterized by recurrent inflammatory episodes of cartilaginous structures and the involvement of special sense organs. The diagnostic criteria of McAdam 1976 include at least three of the following criteria: a) bilateral auricular chondritis, b) nonerosive sero-negative inflammatory polyarthritis, c) nasal chondritis, d) ocular inflammation, e) respiratory tract chondritis, f) audiovestibular chondritis. A cartilage biopsy according to these criteria is not mandatory. Nevertheless, unclear cases still remain as there is a broad spectrum of differential diagnosis. In these individuals it is important to obtain a biopsy from the affected cartilage. Although up to 89% develop auricular inflammation, only few electron microscope studies are performed on cartilage specimens. The purpose of this study is to report on a patient with a history of recurrent swelling of both ears, where the diagnosis could only be established by ear biopsy which was studied by light and electron microscopy. Differential diagnosis is discussed and a review of the literature is given.

Topics: Biopsy; Cartilage Diseases; Diagnosis, Differential; Ear Cartilage; Humans; Inflammation; Male; Middle Aged; Nose; Polychondritis, Relapsing

Topics: Adult; Cartilage; Cyclosporine; Dapsone; Ear Cartilage; Female; Humans; Nose; Polychondritis, Relapsing

Four rare cases of congenital saddle-nose deformity and slowly progressive degeneration of laryngeal cartilages with stenosis are described. The term inherited degenerative chondropathy is suggested for this disease entity. To our knowledge this is the first article on such a disease.

Topics: Adolescent; Child; Female; Humans; Laryngeal Diseases; Laryngostenosis; Male; Nose; Nose Diseases; Pedigree; Polychondritis, Relapsing

Topics: Aged; Airway Obstruction; Ear; Humans; Inflammation; Male; Nose; Polychondritis, Relapsing; Skin Diseases; Trachea

Relapsing polychondritis (RP) is a rare disease characterized by auricular, nasal and respiratory tract chondritis, ocular inflammation, inflammatory polyarthritis and cardiovascular abnormalities. Here we describe a patient with a five-year history of mild nasal and auricular chondritis, which suddenly developed into a severe aortic insufficiency with rest dyspnea. The pathogenesis and the management of this rare connective tissue disease are discussed.

Topics: Adult; Aortic Valve Insufficiency; Dyspnea; Ear, External; Heart Block; Humans; Male; Nose; Polychondritis, Relapsing; Tachycardia; Ultrasonography

Topics: Cartilage; Humans; Nose; Polychondritis, Relapsing; Prednisone

With the help of a casuistic and the results of own investigations clinical, differential-diagnostic, biochemical, pathogenetic and therapeutic aspects of panchondritis systemica are described. The very rare clinical picture, which coincides with chondrolysis and perichondritis, manifests itself intermittently in the region of the auricles, the nose, the cartilaginous parts of the ribs as well as of the chondroskeleton of the respiratory system and in most cases has a fatal termination. Probably on the basis of the pathogenetically decisive changes of the basic substance in addition to this arthritic symptoms, symptoms similar to Bechterew's disease, carditic as well as ocular and internal ear symptoms may appear. Results of own investigations refer to considerable abnormalities in the glucosamine glycan metabolism, in which case the question must remain open, whether these biochemical and immunologic factors are to be regarded as the primary pathogenetic cause. For avoiding irreparable and dangerous defects of the cartilage a sufficiency dosed treatment with corticoids is necessary.

Topics: Adult; Creatinine; Ear, External; Female; Glycosaminoglycans; Humans; Nose; Polychondritis, Relapsing; Prednisolone; Spine; Uronic Acids

Topics: Animals; Antibodies; Antibodies, Anti-Idiotypic; Antibodies, Antinuclear; Arthritis, Rheumatoid; Binding Sites, Antibody; Cartilage; Cattle; Diabetes Complications; Ear; False Positive Reactions; Female; Fluorescent Antibody Technique; Humans; Insulin Resistance; Lupus Erythematosus, Systemic; Middle Aged; Neutralization Tests; Nose; Polychondritis, Relapsing; Rabbits; Rats; Trachea

Topics: Aorta, Thoracic; Aortic Valve Insufficiency; Cartilage Diseases; Ear; Humans; Male; Middle Aged; Nose; Polychondritis, Relapsing; Urethritis

Topics: Cartilage; Cortisone; Diagnosis, Differential; Ear Deformities, Acquired; Ear, External; Humans; Inflammation; Nose; Nose Deformities, Acquired; Pathology; Polychondritis, Relapsing; Prednisone