BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare,life-threatening disorder caused by abnormal histiocytes and T cell activation.In adults,it is predominantly associated with infections,cancers,and autoimmun...BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare,life-threatening disorder caused by abnormal histiocytes and T cell activation.In adults,it is predominantly associated with infections,cancers,and autoimmune diseases.Relapsing polychondritis(RP),another rare disease,is diagnosed based on symptoms without specific tests,featuring cartilage inflammation characterized by swelling,redness,and pain,rarely inducing HLH.CASE SUMMARY A 74-year-old woman visited the emergency room with a fever of 38.6℃.Blood tests,cultures,and imaging were performed to evaluate fever.Results showed increased fluorescent antinuclear antibody levels and mild cytopenia,with no other specific findings.Imaging revealed lymph node enlargement was observed;however,biopsy results were inconclusive.Upon re-evaluation of the physical exam,inflammatory signs suggestive of RP were observed in the ears and nose,prompting a tissue biopsy for confirmation.Simultaneously,persistent fever accompanied by cytopenia prompted a bone marrow examination,revealing hemophagocytic cells.After finding no significant results in blood culture,viral markers,and tissue examination of enlarged lymph nodes,HLH was diagnosed by RP.Treatment involved methylprednisolone followed by azathioprine.After two months,bone marrow examination confirmed resolution of hemophagocytosis,with normalization of hyperferritinemia and pancytopenia.CONCLUSION Thorough physical examination enabled diagnosis and treatment of HLH trig gered by RP in patients presenting with fever of unknown origin.展开更多
Relapsing polychondritis(RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly ...Relapsing polychondritis(RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody(ANCA) associated vasculitis(AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia,which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease.展开更多
Relapsing polychondritis is a rare inflammatory disease involving essentially cartilaginous structures. Other systemic manifestations can be encountered as the eye and ear disturbance. Aortic aneurysms affect few case...Relapsing polychondritis is a rare inflammatory disease involving essentially cartilaginous structures. Other systemic manifestations can be encountered as the eye and ear disturbance. Aortic aneurysms affect few cases. If affection of airway cartilages occurs, prognosis may be worsened by eventual stenotic lesions. We report a 22-year-old woman with Relapsing polychondritis. When she was referred to our hospital 4 weeks after the onset of respiratory symptoms, she was having severe breathing difficulty. Immediate tracheostomy followed by steroid therapy improved her respiratory condition, although the treatment was complicated because of her diabetes. While airway involvement of Relapsing polychondritis can be life threatening, it is curable with steroid therapy. Clinicians should keep in mind that airway obstruction could be caused by this disease.展开更多
BACKGROUND Relapsing polychondritis is a rare multisystem autoimmune disease that mainly involves systemic cartilage and proteoglycan-rich tissues.If the larynx and trachea are involved,the patient’s condition deteri...BACKGROUND Relapsing polychondritis is a rare multisystem autoimmune disease that mainly involves systemic cartilage and proteoglycan-rich tissues.If the larynx and trachea are involved,the patient’s condition deteriorates rapidly.When relapsing polychondritis becomes more advanced,the airways collapse and treatment is difficult,rendering a poor prognosis.Therefore,the diagnosis method,treatment strategy and prognosis of relapsing polychondritis with larynx and trachea involvement need to be elucidated to improve clinicians’awareness of the disease.CASE SUMMARY A man and a woman were admitted because of breathlessness.Relapsing polychondritis was diagnosed after a series of accessory examinations.They were both treated with glucocorticoids and immunosuppressants,and underwent tracheotomy as their breathing difficulties could not be relieved by the medication.CONCLUSION The two cases highlight the importance of the timely diagnosis,full evaluation and initiating individualized treatment of relapsing polychondritis with larynx and trachea involvement.Laryngoscopy,bronchoscopy and pathological examination are helpful in diagnosis of this disease.展开更多
Background: Exercise training has demonstrating to be safe and promote benefits for several rheumatologic autoimmune diseases. However, no study has evaluated the safety and benefits of exercise in relapsing polychond...Background: Exercise training has demonstrating to be safe and promote benefits for several rheumatologic autoimmune diseases. However, no study has evaluated the safety and benefits of exercise in relapsing polychondritis. Aim: To evaluate the effectiveness of an exercise training program in a patient with relapsing polychondritis. Case presentation: A 67-year-old female patient with relapsing polychondritis in remission was submitted to a 12-week, twice weekly, aerobic and resistance training program. Aerobic capacity, muscle strength and function capacity, as well as body composition, were evaluated at baseline and after 12-weeks. Conclusions: Exercise training program demonstrated to be effective for increasing aerobic capacity, muscle strength and function, and for improving body composition in the patient. Further studies are necessary to confirm these findings.展开更多
The incidence of Relapsing Polychondritis is estimated to be 3.5 cases per million with only 600 cases being reported in the world. It can affect any age ranging from 5 to 84 years with predominance in the fourth and ...The incidence of Relapsing Polychondritis is estimated to be 3.5 cases per million with only 600 cases being reported in the world. It can affect any age ranging from 5 to 84 years with predominance in the fourth and fifth decade. Male to female ratio varies from 1:1 to 1:3. It is believed to be an immunologic reaction to collagen Type II which is predominantly presented in the cartilaginous structures of the body and in the eye. The diagnosis is based on the presence of three or more clinical signs, one clinical sign in addition to histological confirmation, or involvement of two or more sites with a favorable response to treatment based on Mc Adam diagnostic criteria. The otolaryngologic manifestations of RP span the ears, nose, larynx and tracheobronchial tree. The symptoms include change in voice quality, respiratory discomfort, stridor, dyspnea, cough, chocking, anterior neck tenderness mainly over the thyroid cartilage and cricoid cartilages, aspiration and difficulty in swallowing. The laryngeal manifestations should be evaluated using high resolution computerized tomography, Magnetic resonance imaging, pulmonary function testing, plain radiography and pulmonary function testing.展开更多
Relapsing polychondritis is a rare cartilaginous inflammatory disease affecting the external ear, nose, peripheral joints and tracheobronchial tree. It is characterized by recurrent inflammation and degeneration of ca...Relapsing polychondritis is a rare cartilaginous inflammatory disease affecting the external ear, nose, peripheral joints and tracheobronchial tree. It is characterized by recurrent inflammation and degeneration of cartilage and connective tissue. A 72-year-old man complained of dyspnea, cough and wheezing for 2 months. Diffuse wall thickening and narrowing from the trachea to segmental bronchus were seen on chest CT. Tracheostomy was performed in order to avoid as-phyxia, and he was diagnosed as relapsing polychondritis on the basis of pathology evaluation of a tracheal biopsy specimen. He was treated with high doses of a glucocorticoid, with which his symptoms improved. However, the cough and wheezing recurred after tapering of the glucocorticoid. His symptoms thereafter were improved by combination of the glucocorticoid with cyclosporine. The immunosuppressive agent provided effective treatment for glucocorticoid-resistant relapsing polychondritis.展开更多
BACKGROUND Relapsing polychondritis(RP)is a rare inflammatory disease involving the systemic cartilage,such as the auricle,trachea,and bronchiole,among others.A patient with RP shows variable symptoms based on the inv...BACKGROUND Relapsing polychondritis(RP)is a rare inflammatory disease involving the systemic cartilage,such as the auricle,trachea,and bronchiole,among others.A patient with RP shows variable symptoms based on the involved cartilage.CASE SUMMARY A 72-year-old Japanese woman with a history of redness of the bilateral auricles for 3 d was referred to a clinician.The clinician prescribed antibiotics to the patient;however,the symptoms worsened;thus,she was referred to our hospital.Head and neck magnetic resonance imaging(MRI)showed edematous auricle with remarkable contrast,fluid collection in the bilateral mastoid cells,suggesting otitis media.The eustachian tube(ET)on the right side was also edematous with contrast enhancement.The patient was suspected of RP according to the diagnostic criteria.A biopsy of the auricular cartilage was performed by an otorhinolaryngologist,confirming pathological proof of RP.Treatments with steroids were immediately administered thereafter.CONCLUSION We highlight a rare case of RP with radiologically confirmed involvement of ET in the MRI.展开更多
BACKGROUNDRelapsing polychondritis (RP) is a rare, long-term, and potentially life-threateningdisease characterised by recurrent paroxysmal inflammation that can involve anddestroy the cartilage of the external ear, n...BACKGROUNDRelapsing polychondritis (RP) is a rare, long-term, and potentially life-threateningdisease characterised by recurrent paroxysmal inflammation that can involve anddestroy the cartilage of the external ear, nose, larynx, and trachea.CASE SUMMARYWe here report a case of RP involving solely the tracheobronchial cartilage ring(and not the auricular. nasal or articular cartilage) complicated by Sjögren's syndrome in a 47-year-old female whose delayed diagnosis caused a sharpdecline in pulmonary function. After corticosteroid treatment, her pulmonaryfunction improved.CONCLUSIONIn such cases, our experience suggested that 18F-fluorodeoxyglucose positronemission tomography/computed tomography (18F-FDG PET/CT) and fiberopticbronchoscopy should be used to diagnose airway chondritis as relapsing polychondritisin the early phase of disease.展开更多
Relapsing polychondritis （RP） is a recurrent disease involving cartilage mainly of the ear,nose, larynx, trachea, and bronchus. The typical manifestations of the disease in the ear and nose can be easily recognized,...Relapsing polychondritis （RP） is a recurrent disease involving cartilage mainly of the ear,nose, larynx, trachea, and bronchus. The typical manifestations of the disease in the ear and nose can be easily recognized, but the symptoms could be ignored or easily confused with those of other diseases when the cartilage of other sites is involved. Thus, it is necessary to develop a new technique for the diagnosis of this disease. Few cases of abnormal accumulation of radioactivity at cartilage shown by ^99mTc methylene diphosphonate （MDP） bone scintigraphy are described in the literature. In this report, we present 4 patients of whom 3 had positive findings on ^99mTc MDP bone scintigraphy with an assessment of ^99mTc MDP bone scintigraphy in the diagnosis of RP.展开更多
Relapsing polychondritis （RP） is a rare disorder of unknown cause. It is characterized by recurrent inflammation of cartilage and connective tissue. Airway complications are the most serious manifestations. The anes...Relapsing polychondritis （RP） is a rare disorder of unknown cause. It is characterized by recurrent inflammation of cartilage and connective tissue. Airway complications are the most serious manifestations. The anesthetic management of patients with RP is challenging Airway management is of primary importance because of the potential for collapse of supporting airway structures with resulting inability to intubate and ventilate the patient. Although it tends to occur in middle age （〉 40 years）, it has been reported in younger individuals. Herein we report a case of anesthetic management of a child with RP for tracheotomy combined with insertion of T tube stent and review several other cases.展开更多
Background:To report rapid corneal thinning and perforation in a case with relapsing polychondritis.Case presentation:A 43 year-old male diagnosed with relapsing polychondritis suffered from bilateral scleritis,bilate...Background:To report rapid corneal thinning and perforation in a case with relapsing polychondritis.Case presentation:A 43 year-old male diagnosed with relapsing polychondritis suffered from bilateral scleritis,bilateral swelling of pinna,saddle nose and tracheal stenosis.The patient presented with right eye pain and redness for one month.Slit lamp examination of the right eye showed 80%peripheral corneal thinning between 3 and 7 o’clock.The best-corrected visual acuity(BCVA)was 1.0 bilaterally.The degree of corneal thinning worsened to 90%after one week of oral corticosteroid use.Subsequently,topical cyclosporine 2%eye drops four times a day,oral doxycycline 100 mg twice a day and oral vitamin C 2 g daily were added.The corneal thinning gradually improved to about 60%.However,the patient rapidly tapered oral prednisolone against medical advice and returned with an acute drop in vision in his right eye.Slit lamp examination of the right eye showed peripheral corneal perforation with iris prolapse.An emergency repair with cyanoacrylate glue was performed.Intravenous methylprednisolone 1 mg/kg body weight was administered for three days and 1 g/day intravenous immunoglobulin was administered every four weeks.At 3 months postoperatively,BCVA in the right eye was 0.6.Slit lamp examination showed a well-formed anterior chamber with glue in situ.Conclusions:Relapsing polychondritis may be associated with rapid corneal thinning.The clinicians should be aware of the possibility of corneal perforation in these cases.Cyanoacrylate glue is a viable temporary management option in such scenarios.展开更多
文摘BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare,life-threatening disorder caused by abnormal histiocytes and T cell activation.In adults,it is predominantly associated with infections,cancers,and autoimmune diseases.Relapsing polychondritis(RP),another rare disease,is diagnosed based on symptoms without specific tests,featuring cartilage inflammation characterized by swelling,redness,and pain,rarely inducing HLH.CASE SUMMARY A 74-year-old woman visited the emergency room with a fever of 38.6℃.Blood tests,cultures,and imaging were performed to evaluate fever.Results showed increased fluorescent antinuclear antibody levels and mild cytopenia,with no other specific findings.Imaging revealed lymph node enlargement was observed;however,biopsy results were inconclusive.Upon re-evaluation of the physical exam,inflammatory signs suggestive of RP were observed in the ears and nose,prompting a tissue biopsy for confirmation.Simultaneously,persistent fever accompanied by cytopenia prompted a bone marrow examination,revealing hemophagocytic cells.After finding no significant results in blood culture,viral markers,and tissue examination of enlarged lymph nodes,HLH was diagnosed by RP.Treatment involved methylprednisolone followed by azathioprine.After two months,bone marrow examination confirmed resolution of hemophagocytosis,with normalization of hyperferritinemia and pancytopenia.CONCLUSION Thorough physical examination enabled diagnosis and treatment of HLH trig gered by RP in patients presenting with fever of unknown origin.
基金Supported by Department of Nephrology,University of Debrecen,Clinical Centre,Debrecen,HungaryThe project implemented through the New Hungary Development Planco-financed by the European Union and the European Social Fund,No.TáMOP4.2.2.A-11/1/KONV-2012-0045
文摘Relapsing polychondritis(RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody(ANCA) associated vasculitis(AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia,which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease.
文摘Relapsing polychondritis is a rare inflammatory disease involving essentially cartilaginous structures. Other systemic manifestations can be encountered as the eye and ear disturbance. Aortic aneurysms affect few cases. If affection of airway cartilages occurs, prognosis may be worsened by eventual stenotic lesions. We report a 22-year-old woman with Relapsing polychondritis. When she was referred to our hospital 4 weeks after the onset of respiratory symptoms, she was having severe breathing difficulty. Immediate tracheostomy followed by steroid therapy improved her respiratory condition, although the treatment was complicated because of her diabetes. While airway involvement of Relapsing polychondritis can be life threatening, it is curable with steroid therapy. Clinicians should keep in mind that airway obstruction could be caused by this disease.
文摘BACKGROUND Relapsing polychondritis is a rare multisystem autoimmune disease that mainly involves systemic cartilage and proteoglycan-rich tissues.If the larynx and trachea are involved,the patient’s condition deteriorates rapidly.When relapsing polychondritis becomes more advanced,the airways collapse and treatment is difficult,rendering a poor prognosis.Therefore,the diagnosis method,treatment strategy and prognosis of relapsing polychondritis with larynx and trachea involvement need to be elucidated to improve clinicians’awareness of the disease.CASE SUMMARY A man and a woman were admitted because of breathlessness.Relapsing polychondritis was diagnosed after a series of accessory examinations.They were both treated with glucocorticoids and immunosuppressants,and underwent tracheotomy as their breathing difficulties could not be relieved by the medication.CONCLUSION The two cases highlight the importance of the timely diagnosis,full evaluation and initiating individualized treatment of relapsing polychondritis with larynx and trachea involvement.Laryngoscopy,bronchoscopy and pathological examination are helpful in diagnosis of this disease.
文摘Background: Exercise training has demonstrating to be safe and promote benefits for several rheumatologic autoimmune diseases. However, no study has evaluated the safety and benefits of exercise in relapsing polychondritis. Aim: To evaluate the effectiveness of an exercise training program in a patient with relapsing polychondritis. Case presentation: A 67-year-old female patient with relapsing polychondritis in remission was submitted to a 12-week, twice weekly, aerobic and resistance training program. Aerobic capacity, muscle strength and function capacity, as well as body composition, were evaluated at baseline and after 12-weeks. Conclusions: Exercise training program demonstrated to be effective for increasing aerobic capacity, muscle strength and function, and for improving body composition in the patient. Further studies are necessary to confirm these findings.
文摘The incidence of Relapsing Polychondritis is estimated to be 3.5 cases per million with only 600 cases being reported in the world. It can affect any age ranging from 5 to 84 years with predominance in the fourth and fifth decade. Male to female ratio varies from 1:1 to 1:3. It is believed to be an immunologic reaction to collagen Type II which is predominantly presented in the cartilaginous structures of the body and in the eye. The diagnosis is based on the presence of three or more clinical signs, one clinical sign in addition to histological confirmation, or involvement of two or more sites with a favorable response to treatment based on Mc Adam diagnostic criteria. The otolaryngologic manifestations of RP span the ears, nose, larynx and tracheobronchial tree. The symptoms include change in voice quality, respiratory discomfort, stridor, dyspnea, cough, chocking, anterior neck tenderness mainly over the thyroid cartilage and cricoid cartilages, aspiration and difficulty in swallowing. The laryngeal manifestations should be evaluated using high resolution computerized tomography, Magnetic resonance imaging, pulmonary function testing, plain radiography and pulmonary function testing.
文摘Relapsing polychondritis is a rare cartilaginous inflammatory disease affecting the external ear, nose, peripheral joints and tracheobronchial tree. It is characterized by recurrent inflammation and degeneration of cartilage and connective tissue. A 72-year-old man complained of dyspnea, cough and wheezing for 2 months. Diffuse wall thickening and narrowing from the trachea to segmental bronchus were seen on chest CT. Tracheostomy was performed in order to avoid as-phyxia, and he was diagnosed as relapsing polychondritis on the basis of pathology evaluation of a tracheal biopsy specimen. He was treated with high doses of a glucocorticoid, with which his symptoms improved. However, the cough and wheezing recurred after tapering of the glucocorticoid. His symptoms thereafter were improved by combination of the glucocorticoid with cyclosporine. The immunosuppressive agent provided effective treatment for glucocorticoid-resistant relapsing polychondritis.
文摘BACKGROUND Relapsing polychondritis(RP)is a rare inflammatory disease involving the systemic cartilage,such as the auricle,trachea,and bronchiole,among others.A patient with RP shows variable symptoms based on the involved cartilage.CASE SUMMARY A 72-year-old Japanese woman with a history of redness of the bilateral auricles for 3 d was referred to a clinician.The clinician prescribed antibiotics to the patient;however,the symptoms worsened;thus,she was referred to our hospital.Head and neck magnetic resonance imaging(MRI)showed edematous auricle with remarkable contrast,fluid collection in the bilateral mastoid cells,suggesting otitis media.The eustachian tube(ET)on the right side was also edematous with contrast enhancement.The patient was suspected of RP according to the diagnostic criteria.A biopsy of the auricular cartilage was performed by an otorhinolaryngologist,confirming pathological proof of RP.Treatments with steroids were immediately administered thereafter.CONCLUSION We highlight a rare case of RP with radiologically confirmed involvement of ET in the MRI.
文摘BACKGROUNDRelapsing polychondritis (RP) is a rare, long-term, and potentially life-threateningdisease characterised by recurrent paroxysmal inflammation that can involve anddestroy the cartilage of the external ear, nose, larynx, and trachea.CASE SUMMARYWe here report a case of RP involving solely the tracheobronchial cartilage ring(and not the auricular. nasal or articular cartilage) complicated by Sjögren's syndrome in a 47-year-old female whose delayed diagnosis caused a sharpdecline in pulmonary function. After corticosteroid treatment, her pulmonaryfunction improved.CONCLUSIONIn such cases, our experience suggested that 18F-fluorodeoxyglucose positronemission tomography/computed tomography (18F-FDG PET/CT) and fiberopticbronchoscopy should be used to diagnose airway chondritis as relapsing polychondritisin the early phase of disease.
文摘Relapsing polychondritis （RP） is a recurrent disease involving cartilage mainly of the ear,nose, larynx, trachea, and bronchus. The typical manifestations of the disease in the ear and nose can be easily recognized, but the symptoms could be ignored or easily confused with those of other diseases when the cartilage of other sites is involved. Thus, it is necessary to develop a new technique for the diagnosis of this disease. Few cases of abnormal accumulation of radioactivity at cartilage shown by ^99mTc methylene diphosphonate （MDP） bone scintigraphy are described in the literature. In this report, we present 4 patients of whom 3 had positive findings on ^99mTc MDP bone scintigraphy with an assessment of ^99mTc MDP bone scintigraphy in the diagnosis of RP.
文摘Relapsing polychondritis （RP） is a rare disorder of unknown cause. It is characterized by recurrent inflammation of cartilage and connective tissue. Airway complications are the most serious manifestations. The anesthetic management of patients with RP is challenging Airway management is of primary importance because of the potential for collapse of supporting airway structures with resulting inability to intubate and ventilate the patient. Although it tends to occur in middle age （〉 40 years）, it has been reported in younger individuals. Herein we report a case of anesthetic management of a child with RP for tracheotomy combined with insertion of T tube stent and review several other cases.
文摘Background:To report rapid corneal thinning and perforation in a case with relapsing polychondritis.Case presentation:A 43 year-old male diagnosed with relapsing polychondritis suffered from bilateral scleritis,bilateral swelling of pinna,saddle nose and tracheal stenosis.The patient presented with right eye pain and redness for one month.Slit lamp examination of the right eye showed 80%peripheral corneal thinning between 3 and 7 o’clock.The best-corrected visual acuity(BCVA)was 1.0 bilaterally.The degree of corneal thinning worsened to 90%after one week of oral corticosteroid use.Subsequently,topical cyclosporine 2%eye drops four times a day,oral doxycycline 100 mg twice a day and oral vitamin C 2 g daily were added.The corneal thinning gradually improved to about 60%.However,the patient rapidly tapered oral prednisolone against medical advice and returned with an acute drop in vision in his right eye.Slit lamp examination of the right eye showed peripheral corneal perforation with iris prolapse.An emergency repair with cyanoacrylate glue was performed.Intravenous methylprednisolone 1 mg/kg body weight was administered for three days and 1 g/day intravenous immunoglobulin was administered every four weeks.At 3 months postoperatively,BCVA in the right eye was 0.6.Slit lamp examination showed a well-formed anterior chamber with glue in situ.Conclusions:Relapsing polychondritis may be associated with rapid corneal thinning.The clinicians should be aware of the possibility of corneal perforation in these cases.Cyanoacrylate glue is a viable temporary management option in such scenarios.
