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Auricular chondritis frequently occurs in relapsing polychondritis. In addition to the primary form of the disease up to 30% of cases of chondritis can be secondary, e.g. due to autoimmune diseases. We describe the case of a 62-year-old male patient with auricular chondritis as the first symptom of granulomatosis with polyangiitis. Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis should always be considered in the differential diagnostics of relapsing polychondritis and antibody testing should be performed accordingly.

Relapsing polychondritis (RPC) is a chronic immune-mediated inflammatory, systemic disease primarily leading to structural damage and impaired function of cartilage tissue. However, the systemic inflammatory process in RPC can also affect sensory organ structures, the respiratory tract, the nervous and cardiovascular systems as well as the kidneys. The immune-mediated disease leads to recurrent inflammatory attacks causing a progressive degradation of elastic and hyaline cartilage structures, especially in the ears, nose, larynx, trachea and diarthrodial joints. However, other connective tissue structures in the eye and the heart valves may also be involved. The RPC is regarded as an orphan disease as the number of reported cases has so far remained confined to approximately 600 worldwide. The rarity of the disease has limited systematic clinical studies and the available empirical data are exclusively derived from casuistic studies or evaluation of small case series. The therapeutic interventions depend on the extent and localization of the disease manifestation. Thus, nonsteroidal anti-inflammatory drugs (NSAID), glucocorticoids and immunosuppressive agents with conventional synthetic disease-modifying antirheumatic drugs (DMARD) have been demonstrated to be beneficial. More severe and refractory diseases may require a targeted pharmacological intervention with biologic DMARDs.

This article presents an overview of the typical clinical and imaging features of relapsing polychondritis (RP), a rare autoimmune disease, based on a case reportAlthough the diagnosis is mainly established clinically, the use of (18)F fluorodeoxyglucose positron emission tomography-computed tomography ((18)F-FDG-PET/CT) has been proven to be a useful diagnostic tool to accurately determine the extent of inflammation throughout the body [corrected]. Furthermore (18)F-FDG-PET/CT provides a high sensitivity for detection of a relapse, especially when clinical and laboratory results are inconclusive. Additionally, (18)F-FDG-PET/CT helps to assess disease activity during the course of therapy.

Chronic inflammatory diseases including tuberculosis, rheumatic disorders (rheumatoid arthritis, sarcoidosis, systemic lupus erythematosus, Wegeners's granulomatosis, relapsing polychondritis) and reflux disease are considered as systemic diseases, and may affect the larynx. The clinical symptoms are often unspecific, leading to prolonged intervals to diagnosis. Solid and haematological tumours should be considered in differential diagnosis and may require bioptic sampling. Treatment may require interdisciplinary approach.

We report on the case of a 63-year-old patient with a meningoencephalitis, presenting itself with headache and a general neuropsychological retardation. Additionally, a reddening and swelling of both auricles could be seen. Magnetic resonance imaging showed confluent, contrast-uptaking lesions. In the cerebrospinal fluid an aseptic lymphocytic pleocytosis was found. A biopsy of the ear revealed a chronic lymphoplasmacellular inflammatory reaction. We diagnosed a relapsing polychrondritis, an inflammatory disease of the cartilage, which can in rare cases affect the central nervous system. Typically, the patients complain about red swollen ears, the "red puffy ear sign". After initiation of steroid and azathioprin therapy the patient recovered fully.

Relapsing polychondritis is a rare autoimmune disease associated with inflammation and destruction of cartilage and connective tissue.We report on a patient with a severe form of this disease that had a progressive and complicated course despite administration of a number of disease-modifying anti-rheumatic drugs.Finally, therapy with the TNF-alpha-antagonist etanercept was initiated, which led to a considerable decrease in disease activity. This case is further evidence for the efficiency of TNF-alpha-antagonists in relapsing polychondritis.

A 76-year old patient with painful erythematous swelling of the right ear was initially treated with antibiotics under the suspected diagnosis of erysipelas. Her failure to respond and a history of previous laryngeal and nasal swelling suggested the possibility of relapsing polychondritis. High dose prednisolone therapy produced marked improvement. Relapsing polychondritis should be considered as a rare, but important differential diagnostic consideration for erysipelas.

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.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.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.

We report about a 43-year old woman with relapsing polychondritis, admitted with progressive malaise, fatigue, anorexia and profound weight loss. Two years ago a nasal chondritis with characteristic changes of the nasal profil, scleritis, laryngitis and unspecific general symptoms (fever, fatigue, signs of a systemic inflammation) developed and relapsing polychondritis was diagnosed. The patient was treated initially with azathioprine followed by methotrexate in combination with ciclosporine and glucocorticoids. During the hospitalization her condition slowly worsened, and crampy abdominal pain developed subsequently with massive tenderness, rebound and guarding. The clinically presumed diagnosis of ileus and peritonitis was radiomorphologically confirmed with signs of enteric perforation and a laparatomy was performed. A complete occlusion of the A. mesenterica superior and stenosis of the truncus coeliacus were diagnosed followed by a revascularization with an aorto-mesenteric bypass and subsequent resection of the necrotic ischemic ileum. An association with various autoimmune disorders including vasculitic syndromes is well known for relapsing polychondritis. The case demonstrated a progressive mesenteric ischemia with the acute exacerbation caused by a vasculitic mesenterial occlusion. The unusual presentation should be considered in the differential diagnosis of uncommon abdominal symptoms during the course of relapsing polychondritis.

We report the case of a 41-year-old female patient with relapsing polychondritis and severe respiratory involvement. The patient presented with acute respiratory failure requiring endotracheal intubation. Bronchoscopy revealed tracheal collapse and inflammatory stenoses with dynamic collapse of the major airways. We describe a multidisciplinary therapeutic approach, consisting of immunosuppressive treatment, bronchoscopic placement of self-expandable stents into the collapsing bronchi, and tracheotomy. In addition, we report the effectiveness of oral cyclophosphamide for treatment of relapsing polychondritis with severe respiratory involvement after failure of other immunosuppressive agents. The problem of severe respiratory complications in patients with relapsing polychondritis and the need for a multidisciplinary approach is discussed.

A 68-years old patient with relapsing polychondritis of the ear developed stenosis and occlusion of hercarotid and femoropopliteal arteries. Relapsing polychondritis is a rare systemic disorder which may take a fetal course. We also review this rare, systemic disorder.

We report on a 71-year-old female patient with relapsing polychondritis in both ears. Monotherapy with low-dose ciclosporin led to a complete remission.

A 62-year-old male had tender swelling of both ears with loss of hearing due to edematous swelling of the external auditory canal. The patient complained further about dry cough, pain at the costo-sternal junctions, adynamia and weight loss. Inflammatory parameters were markedly elevated, and histologic work-up of an auricular biopsy revealed lymphocytic infiltration. These findings led to the diagnosis of chronic recurrent polychondritis actually under control after a pulse of glucocorticoids followed by maintenance therapy with 5 mg prednisone.

Relapsing polychondritis is a rare systemic disease. Main symptoms are recurrent inflammations of the cartilage tissues, the eyes, and the cardiovascular system. Etiology and pathogenesis are obscure, there are hints for an immunologic disorder.Our patient has suffered from the disease since the age of 12 years. The patient has experienced recurrent keratitis with spontaneous ulcerations for 15 years.

Chronic relapsing polychondritis is a rare connective tissue disease of presumed autoimmunologic pathogenesis. It may involve multiple organ systems. The most characteristic signs are: relapsing inflammation of the cartilage of the outer ear, non-erosive polyarthritis, chondritis of the nasal cartilage, inflammation of different ocular tissues, inflammation of tracheal and bronchial cartilages and lesions of the inner ear. The wide range of ocular tissue involvement is discussed on the basis of two new cases with emphasis on scleritis, episcleritis, keratitis and chorioretinal involvement. Ocular disease complications that have not been published before are the massive development of subretinal stands following multiple intra- and subretinal infiltrates in the posterior pole connecting areas of chorioretinal scars. The importance of high-dose, long-term steroid therapy is stressed. In addition to steroids, immunosuppressive agents such as azathioprine and cyclophosphamide are sometimes mandatory to cope with severe multi-organ disease. Another option in very severe relapses may be plasma separation to improve the condition rapidly until drug therapy can be effective.

A survey of literature dealing with relapsing polychondritis is given hinting at an autoimmune etiology. Two cases with mainly laryngotracheal symptoms are discussed. A new hypothesis about pathogenesis has not been found.

For three months a 17-year-old boy had been suffering from laryngitis, pharyngitis and conjunctivitis, weight loss, nocturnal sweating and signs of changing cranial-nerve involvement (dizziness, nausea, nystagmus). The diagnosis of recurrent polychondritis was made only when, in addition to a definite inspiratory stridor there also developed a painful swelling of the left ear cartilage and a saddle nose due to loss of the cartilaginous portion of the nasal skeleton. Histological examination of the inflamed ear cartilage confirmed the diagnosis. After treatment with prednisone (100 mg daily, gradually reduced to 25 mg) and azathioprine (100 mg daily, increased to 150 mg after three weeks), there was clinical improvement, but the airway resistance (R = 6.2 cm H2O.s/l) rose further, requiring tracheostoma twelve months after onset of symptoms.

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.

Relapsing polychondritis is a rare disease of unknown etiology. The symptomatology is characterised by inflammatory and degenerative changes of multiple cartilaginous structures. --In this peculiar case the nose, the larynx and the cartilages of the ribs and of one hand were involved. Distinct inflammatory changes of the left vocal cord were seen. The diagnosis of the disease was assured by the presented electron microscopic studies. Treatment with high doses of corticosteroids did not prove effective. The disease process could only be controlled by in immunosuppressive combination therapy with prednisone, and cyclophosphamide. --Characteristic clinical features, diagnosis, pathogenesis and therapeutical problems are discussed.

Four patients with relapsing polychondritis and eye disease were observed. Diagnosis was made on the basis of clinical symptoms of multifocal chondritis and episcleritis in one case. In the latter patient the disease started with slight visual impairment due to retinal hemorrhage and edema. Despite a relapsing course all patients eventually responded well to topical and systemic corticosteroids. Immunopathologic events are discussed.

Relapsing polychondritis is a rare, at times fatal systemic disease involving inflammatory destruction mainly of cartilaginous structures. The sensory organs, respiratory tract, cardiovascular system and kidneys are most frequently involved. Based on the current state of knowledge it seems probable that relapsing polychondritis is an autoimmune disease. Early involvement of the respiratory tract and kidneys, anemia, arthritis and saddle-nose deformity are associated with poor prognosis. 2 cases of relapsing polychondritis (one with erosive symmetrical polyarthritis and the second with massive involvement of the respiratory tract) are presented.

Report on a patient suffering from relapsing polychondritis, who showed the cardinal manifestations on ears, eyes and joints, as well as a leucocytoclastic vasculitis and erythema nodosum-like lesions. Corticosteroids (prednisone 50 mg daily) have been effective in controlling all clinical symptoms.

Report about a case of relapsing polychondritis in a woman aged 60 years, which was proofed through autopsy. There was involvement of the trachea, the bronchial system and the cartilages of the ribs. The cartilages of nose and ear did not show any typical changes; therefore the diagnosis could not be established during life. The pecularities of the clinical picture and the pathohistological findings are reported in detail. It seems that the polychondritis is related in it's pathogenesis to the rheumatic disorders. tthe etiology, however, remains obscure.

Relapsing polychondritis is a rare disorder of unknown etiology. It is characterized by arthropathy, inflammation of auricular and nasal cartilage, and various ocular complications. Potentially destructive inflammatory lesions involve cartilaginous structures and the cardiovascular system. In three reported cases multiple ocular complications and arthropathy were early symptoms of relapsing polychondritis.

Report on a 56-year-old woman with relapsing polychondritis, with ocular involvement in the form of bilateral optic atrophy and paresis of the extraocular muscles. The cause of this disorder has not yet been identified, although it is presumed to be an immune disease. Clinically there are some obvious similarities to systemic vasculitis.

A typical case of chronic atrophic polychondritis with chondritis of the ear and episcleritis is presented. The inflammation subsided on treatment with colchicine. This entity is rare. It has an uncertain prognosis and causes a wide variety of symptoms. Accordingly, the spectrum of conditions that need to be considered in the differential diagnosis is also wide.

Basing on the description of a case treated by the authors, the von Meyenburg-Altherr-Uehlinger syndrome is illustrated with its manifold symptoms and the resulting difficulties in diagnosis. Besides the signs of involvement of the eyes, joints, heart and vascular system, the symptoms caused by affection of the cartilage of the ear, nose, trachea and bronchi are important for the ENT surgeon. Aspects of differential diagnosis are discussed. The aetiology and pathogenesis of the syndrome are still unknown. Therapy is symptomatical with steroids and, in special cases, with immunosuppressive drugs.

Relapsing polychondritis is a rare episodic systemic disorder characterised by recurrent, widespread, and potentially destructive inflammatory lesions involving cartilaginous structures, the cardiovascular system, and organs of special sense, such as the eyes and ears. Although the aetiology is unknown and its pathogenesis is incompletely understood, experimental evidence strongly suggests that immunological mediated mechanisms are operative. The following case report adds some considerations to the discussion about the immunopathology of the inner ear.