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
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
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
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
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
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
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.
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.
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.
