| Objective:To explore the clinical characteristics, diagnosis and prognosis of relapsing polychondritis (RP) for its early diagnosis and treatment.Methods:A retrospective analysis was performed for the clinical data for one case diagnosed with RP. We had a literature review from database of CNKI and pubmed at home and aboard from 2001 to 2015 and focused on 143 cases at home with detailed clinical data reported in the recent 10 years to investigate the clinical characteristics, diagnosis, treatment and prognosis of RP. The clinical materials above were analyzed by SPSS 19.0.Results:Relapsing polychondritisis a rare condition without exact mechanism. The disease may be variable in its signs and symptoms, resulting in a difficult diagnosis which may leads to delayed recognition for several months, years or decades. Glucocorticoid, immunosuppressant and biological agents are effective in the controlling of RP. The prognosis is varied greatly within patients with RP.1. We reported one case with RP whose ear and kidney were involved. She got better after systematic use of glucocorticoid. The patient stopped taking medicine after discharge by her. She suffered dyspnea, chest congestion and blurred vision in the following days. Unfortunately she didn’t go for a standard treatment. She is still in follow-up.2. Literaturereview:143 cases were analyzed.(1)Sex:75 cases are male, and 68 cases are female. The incidence rate ratio between male and female is 1.10:1.(2)Age:RP may occur at any age. Minimum is 7 years old. Maximum is 82 years old. Median is 46 years old. The average age is 45.05±15.36 years old.(3)Complication:there are 8 cases associated with malignant tumors.23 cases are combined with autoimmune disordersuch as SLE and so on.(4)Clinical manifestations:Mono-systematic injury were occurred in only 23cases while 120 cases were involved with multiple system damage, including ear(103 cases), pulmonary system(99 cases),arthritis(52 cases),nose(49 cases),eye(49 cases),dermatitis(10 cases),fever(56 cases)and so on.(5)Laboratory test:anemia (72/124),leukocytosis(50/124),higher NUE% (41/124),thrombocytosis (17/124),abnormityof urinalysis (15/53),abnormity of hepatic enzymes(9/66),hypoalbuminemia (8/15),hyperlipemia (3/6), abnormality of thyroidal function (3/12), abnormity of autoantibody spectrum (28/114), abnormality of serum immunoglobulin (24/61), abnormality of serum immunoglobulin (24/61), abnormality ofcomplements(9/45), faster ESR (112/139), higher CRP (72/95).(6)Imaging features:In the cases who took HRCT, stenosis of the airway whose thicken wall may suffer fibrosis even calcification.Swelling of ribs and connective tissue can also be found. Through bronchoscope, incomplete cartilage rings,mucosal edema and calcification as well as collapse of cartilage can be seen.Pathological biopsy showed chronic inflammatory change.(7)Pathological change:30 patients accepted pathological biopsy and the section showed the destruction of normal cartilage structure and the loss of basophilia. In the early phase sections demonstratesneutrophil infiltration while the lymphocytes and plasmacyte infiltration appeared later. In the end, the cartilage will be replaced by fibro tissue and granulation tissue.(8)Treatment:143 of them took systemic glucocorticoid, and 55 of them use glucocorticoid alone, while 82 of them took immunosuppressant such as azathioprine. 7 patients accepted glucocorticoidpulse therapy.4 patient got well by use of biologics.(9) Prognosis:5 cases passed away because of respiratory failure and pulmonary infection.Some dozens of patients suffered related complications such as malformation, deafness, blindness and so on.Conclusions:1. Relapsing polychondritismay occur at any age, more often in men than in women. Adults are susceptible to RP.2. RP can accompany with malignant tumors, autoimmune disorder and metabolic diseases.3. The clinical spectrum may vary from mono-system injury to multi-systemic damage even life-threatening manifestations.4. Glucocorticoid, immunosuppressant and biological agents can be used alone or together to control RPbased on the corresponding state of the patients.5. The prognosis is varied greatly within patients and it is crucial to control RP by make an exact diagnosis early and keep on standard treatment. |
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