Predictive value of bone marrow megakaryocyte count for immunotherapeutic response in primary Sjögren's syndrome patients with severe immune thrombocytopenia: A single-center case-control study in China.

OBJECTIVES: To investigate potential predictors of treatment response in primary Sjögren's syndrome (pSS) patients with severe immune thrombocytopenia (ITP), with a focus on bone marrow megakaryocyte (MK) count. METHODS: This case-control study included patients with pSS and severe ITP who were admitted to Peking Union Medical College Hospital and met the 2002 AECG or 2016 American College of Rheumatology / European League Against Rheumatism criteria for SS. Patients who had overlap other connective tissue diseases and with thrombocytopenia that could be explained by other causes were excluded. Severe ITP was defined as platelet count <20 × 109 /L. Response was evaluated at 3 months after treatment. RESULTS: Sixty-eight eligible patients were included: 34 (50%) achieved complete remission (CR), 18 (26%) partial remission (PR) and 16 (24%) were non-responders (NRs). Fewer infections were found in the CR group (24%) than in the PR (50%) and NR (56%) groups (P = 0.04). The MK count (CR 32 vs PR 36 vs NR 4 per slide, P < 0.001) in the NR group was significantly lower than in the other groups. MK count >6.5 per slide predicted good treatment response, with 85.7% sensitivity, 88.1% specificity and 0.866 area under the curve. Logistic regression indicated that patients with more MKs were more likely to respond to immunotherapy (crude odds ratio [OR] 1.45, 95% CI 1.2-2.0, adjusted OR 1.68, 95% CI 1.2-2.7). CONCLUSIONS: MK count predicted response to immunosuppressive treatment in pSS patients with severe ITP. These patients are recommended to have bone marrow aspiration before treatment initiation. Clinicians should be aware of screening for infections during clinical practice.

Associations between family history of psoriatic disease and clinical characteristics on patients with psoriatic arthritis: a nationwide study from the Chinese Registry of Psoriatic Arthritis (CREPAR II).

OBJECTIVES: The study aimed to identify clinical characteristics in Chinese patients with psoriatic arthritis (PsA) with or without a family history of psoriasis and/or PsA. METHODS: Patients with PsA were recruited based on Chinese REgistry of Psoriatic ARthritis (CREPAR) between December 2018 and June 2021. The demographics, clinical information relating to PsA, laboratory variables and comorbidities were collected. The association between family history of psoriatic disease and clinical characteristics on PsA was analysed using logistic regression analysis. RESULTS: Among 1074 eligible patients with PsA, 313 (29.1%) had a family history of psoriasis and/or PsA. Compared with patients without a family history, notably, patients with a family history of psoriasis and/or PsA had an earlier age of onset of psoriasis and PsA, higher proportions of enthesitis and nail involvement, a higher prevalence of positive human leukocyte antigen-B27 (HLA-B27), lower disease activity score 28-erythrocyte sedimentation rate, higher proportions of hyperlipidaemia, lower proportions of hypertension and diabetes. Furthermore, after adjusting for confounding factors, logistic regression analysis demonstrated that a positive family history of psoriasis and/or PsA was associated with more females (OR 1.514, 95% CI 1.088-2.108, p=0.014), earlier age at psoriasis onset (OR 0.971, 95%CI 0.955-0.988, p=0.001), a higher prevalence of HLA-B27 (OR 1.625 95%CI 1.089-2.426, p=0.018), more presence of nail involvement (OR 1.424, 95%CI 1.007-2.013, p=0.046) and enthesitis (OR 1.393, 95%CI 1.005-1.930, p=0.046), a higher proportion of hyperlipidaemia (OR 2.550, 95%CI 1.506-4.317, p=0.001) in PsA patients. CONCLUSIONS: This was first nationwide study to characterize patients with and without a family history of psoriatic disease in China. The findings from the present study revealed that family history of psoriasis and/or PsA had greater effects on disease phenotypes of PsA, especially nail disease and enthesitis.

Identifying risk and prognostic factors in polyarteritis nodosa patients with digital gangrene.

BACKGROUND: Many patients with polyarteritis nodosa (PAN) complicated by digital gangrene have poor outcomes and related research information is limited. Our aim is to identify the associated risk and prognostic factors in PAN patients with digital gangrene. PATIENTS AND METHODS: We conducted a retrospective study of 148 PAN patients admitted to Peking Union Medical College Hospital from Octorber 2001 to December 2018. Forty-seven (31.8%) PAN patients had digital gangrene. The average age was 40.4 ± 17.9 years. RESULTS: The presence of digital gangrene was correlated with current smoking (P = .008, odds ratio [OR] 2.99, 95% CI, 1.33-6.73), eosinophil elevation (P = .003, OR 4.21, 95% CI, 1.62-10.91) and elevated leukocytes (P = .001, OR 4.26, 95% CI, 1.86-9.78). Thirty-two (68.1%) gangrene patients received methylprednisolone pulse therapy and all of these patients were treated with cyclophosphamide. Nine patients suffered irreversible organ injury and 2 died. Survival analysis showed higher serum C-reactive protein (CRP) was associated with poor prognosis in patients with gangrene (log-rank P = 0.042 and generalized Wilcoxon P = .020). CONCLUSIONS: PAN patients with current smoking and eosinophil elevation were more prone to digital gangrene and a high serum CRP level predicted poor outcomes. The CRP level should be efficiently controlled to ensure a good prognosis.

Spectrum of Spondyloarthritis Among Chinese Populations.

PURPOSE OF REVIEW: This review aims to emphasize interesting and important new findings with a focus on the spectrum of spondyloarthritis (SpA) in China. RECENT FINDINGS: Over the past decade, significant advances have been made in the investigation of SpA epidemiology, the exploration of genetic and environmental risk factors, the identification of clinical features, and the updating of treatment protocols in the Chinese population. The prevalence of ankylosing spondylitis (AS) in China is 0.20-0.42%, and the prevalence of HLA-B27 in AS patients is 88.8-89.4%. HLA-B*2704 is the most common subtype in Chinese AS patients, followed by HLA-B*2705. HLA-A*01, more precisely HLA-A*01:01, may be associated with psoriatic arthritis (PsA). Tumor necrosis factor inhibitors and IL-17A inhibitors have been shown to be effective and safe for AS patients in China. Juvenile-onset AS is relatively rare, accounting for only 9.1% of the AS population. The prevalence of arthritis related to inflammatory bowel disease is 6.9 to 7.2%. A Chinese study showed that the most frequently prescribed medication was methotrexate (66.4%). Biological agents were prescribed in only16.4% of patients with PsA. This review summarizes the latest research in the epidemiology, pathogenesis, clinical manifestations, and management of SpA among Chinese populations. Multiple HLA associations with SpA have also been described, and it is hoped that discoveries of such ethnic-specific risk factor(s) and understanding of their pathological mechanisms may potentially lead to newer targeted therapies for the Chinese populations worldwide.

Endocarditis-associated rapidly progressive glomerulonephritis mimicking vasculitis: a diagnostic and treatment challenge.

BACKGROUND: Infective endocarditis (IE)-associated rapidly progressive glomerulonephritis (RPGN) is rarely reported. Sporadic case reports have noted the diagnostic and therapeutic challenge in IE-associated glomerulonephritis because it may masquerade as idiopathic vasculitis. METHODS: Patients with clinical diagnosis of IE-related RPGN in a tertiary hospital in China between January 2004 and May 2021 were identified and retrospectively reviewed. RESULTS: Twenty-four patients with IE-associated RPGN were identified. All patients presented with fever and multiorgan system involvement on top of heart and kidneys, spleen (79%, 19/24), skin (63%, 15/24), lung (33%, 8/24) and nervous system (17%, 4/24). Six of the 24 patients (25%) were initially suspected to have ANCA-associated or IgA vasculitis. Forty-five percent of patients are seropositive for ANCA. Renal histology showed mesangial and/or endocapillary hypercellularity with extensive crescents in most patients. C3-dominant deposition was the predominant pattern on immunofluorescence and pauci-immune necrotising crescentic glomerulonephritis was observed in one case. All patients received antibiotics with or without surgery. Six patients received immunosuppressive therapy before antibiotics due to misdiagnosis and seven patients received immunosuppressive therapy after antibiotics due to persistence of renal failure. Three of the 24 patients died due to severe infection. All the surviving patients had partial or complete recovery of renal function. CONCLUSION: IE-associated RPGN is rare and the differential diagnosis from idiopathic vasculitis can be challenging due to overlaps in clinical manifestations, ANCA positivity and absence of typical presentations of IE. The prognosis is generally good if antibiotics and surgery are not delayed. The decision on introducing immunoruppressive treatment should be made carefully on a case by case basis when kidney function does not improve appropriately after proper anti-infective therapy.Key messagesInfective endocarditis associated RPGN is rare and differentiating it from idiopathic vasculitis can be challenging due to overlap in clinical manifestations, ANCA positivity and occasional absence of typical manifestations of infective endocarditis.Kidney function usually responds to antibiotic therapy alone.Immunosuppressive therapy may be beneficial in carefully selected patients whose kidney function does not improve with antibiotics alone.

Relapse rates and risk factors for unfavorable neurological prognosis of transverse myelitis in systemic lupus erythematosus: A systematic review and meta-analysis.

BACKGROUND: Transverse myelitis (TM) is a rare but severe systemic lupus erythematosus (SLE) manifestation. To date, the prognostic factors for SLE-associated TM have been far less well-studied. There are also controversial data on the association of antiphospholipid antibodies (aPLs), Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score, longitudinal extensive transverse myelitis (LETM), and decreased complement levels with the outcome of TM. We aimed to review the potential prognostic factors and integrate relapse rates of observational studies for SLE-associated TM. METHOD: To review the prognosis for SLE-associated TM, relevant articles published up to July 30, 2021, were comprehensively and systematically identified from PubMed, EMBASE and Web of Science databases. Five studies encompassing 283 patients with SLE-related TM were included in this meta-analysis; raw data were obtained from three studies. RESULTS: The risk factors for unfavorable neurological outcome included demographic features, clinical characteristics, laboratory data, among which a grade of A, B or C on the American Spinal Injury Association Impairment Scale (AIS) at the onset of TM was associated with poor prognosis (OR: 56.05, 95% CI: 6.29-499.25, P < 0.001). The presence of hypoglycorrhachia was also correlated with a worse prognosis (OR: 10.78, 95% CI: 3.74-31.07, P < 0.001). No noticeable correlation was revealed between a poor outcome and positive aPLs and different aPLs profiles (anticardiolipin antibody [aCL], anti-ß2-glycoprotein I (anti-ß2GPI], lupus anticoagulant [LA]). The pooled 1-, 3- and 5-year relapse rates were 22% (95% CI: 0.13-0.31), 34% (95% CI: 0.22-0.47) and 36% (95% CI: 0.14-0.58), respectively. No significant publication bias was found. CONCLUSION: A grade of A, B, or C on the AIS at initial TM and the presence of hypoglycorrhachia were found to be related to a worse prognosis in patients with SLE-associated TM. Notably, aPLs and different aPLs profiles may not suggest poor neurological outcome. The long-term relapse rate of patients with SLE-associated TM was relatively high. We recommend that treatment be stratified based on the initial severity of myelitis. For patients with severe myelitis, early intensive therapy may be initiated as soon as possible.

Clinical characteristics and long-term outcome of patients with gastrointestinal involvement in eosinophilic granulomatosis with polyangiitis.

Objective: This study aims to investigate clinical characteristics, potential risk factors, as well as long-term outcome in EGPA patients with GI involvement. Methods: A total of 94 EGPA patients were included in this cohort study. We retrospectively reviewed the clinical data, treatment, and outcome of 21 EGPA patients with GI involvement and compared them with other 73 EGPA patients without GI involvement. Multivariate logistic regression was used to find potential risk factors associated with GI involvement in EGPA patients. Results: Compared with EGPA patients without GI involvement, EGPA patients with GI involvement had higher level of hs-CRP (65.1 (24.5-138.9) vs. 21.3 (5.7-39.1) mg/L, p=0.005), higher grades of Birmingham vasculitis activity score (BVAS) (20 (13-29.5) vs. 12 (16-19), p=0.022), higher Five Factor Score (FFS) (1 (1-2) vs. 0 (0-1), p<0.001), and were more likely to have weight loss (66.7% vs. 38.4%, p=0.021) at baseline. In EGPA patients with GI involvement, the most common gastrointestinal symptoms were abdominal pain (90.5%) and diarrhea (42.9%). Weight loss was identified as a potential risk factor for GI involvement in EGPA patients (OR = 4.304, 95% CI 1.339-13.841). During follow-up, EGPA patients with GI involvement showed lower 1-year cumulative survival rate (75.2% vs. 100.0%, P <0.0001) and 3-year cumulative survival rate (67.7% vs. 100.0%, P<0.0001), lower long-term remission rate (33.3% vs. 86.3%, P<0.001), but higher 1-year cumulative relapse rate (19.2% vs. 3.8%, P=0.03) and 3-year cumulative relapse rate (54.6% vs. 13.1%, P<0.001) compared with patients without GI involvement. Conclusion: EGPA patients with GI involvement had distinct features from those without GI involvement, including higher hs-CRP level, higher BVAS and FFS scores. EGPA patients with GI involvement showed lower cumulative survival rate, lower long-term remission rate and higher cumulative relapse rate compared with those without GI involvement.

Immunoglobulin G4-Related Disease Accompanied by Primary Myelofibrosis: Case Report.

Immunoglobulin G4-related disease (IgG4-RD) is a heterogeneous autoimmune fibrosing disorder that presents common pathologic features but with unclear etiology. We report a rare case of IgG4-RD accompanied by primary myelofibrosis that eventually transformed into acute myeloid leukemia. A 50-year-old woman suffered from progressive lacrimal and parotid gland enlargement, diaphoresis, and rapid weight loss. Important clinical findings included remarkable leukocytosis, hyperglobulinemia, and splenomegaly. IgG4-RD was confirmed by salivary gland biopsy. Meanwhile, myelofibrosis was diagnosed according to histopathological findings of bone marrow and genetic mutation test of peripheral blood. The patient was on corticosteroid treatment. However, she developed into acute myeloid leukemia (AML) in the 8th month of follow-up. Our case suggested that myeloproliferative neoplasm (MPN) may co-occur with IgG4-RD. Bone morrow aspiration and genetic tests are helpful for throughout evaluation. An active search for hematological malignancies is warranted at diagnosis and during follow-up for patients who present with unexplained leukocytosis, pancytopenia, splenomegaly, or weight loss.

Renal Involvement and HBV Infection Are Common in Chinese Patients With Cryoglobulinemia.

Objectives: This study aimed to describe the main characteristics of Chinese patients with cryoglobulinemia, especially the characteristics of patients with different causes of cryoglobulinemia. Methods: Eighty inpatients diagnosed with cryoglobulinemia from different wards in Peking Union Medical College Hospital were included in this study. Demographic, clinical, biological, and renal pathological data were collected. We analyzed the characteristics of 61 patients with different causes of cryoglobulinemia. Results: Most patients (36/80, 45%) were diagnosed between 40 and 60 years of age. The male: female ratio was 1:1.5. Mixed (II + III) cryoglobulinemia accounted for the majority (43.8%) of cases. Renal involvement (87.5%), cutaneous involvement (57.5%), and fever (27.5%) were the most common clinical manifestations, while other manifestations included serositis and pulmonary and gastrointestinal involvement. The most common renal histopathological pattern was membranoproliferative glomerulonephritis (25/42, 59.5%). The secondary causes of cryoglobulinemia included infectious diseases (26/61, 32.5%), such as hepatitis B virus (HBV) and hepatitis C virus (HCV) infections, and connective tissue diseases (22/61, 27.5%), such as lupus and hematologic tumors (13/61, 16.3%). Patients with hematologic tumors were diagnosed at an older age (P = 0.044) and mostly had type I cryoglobulinemia (P < 0.001). No significant difference in clinical or biological manifestations was found among patients with different causes of cryoglobulinemia. Conclusions: This is the largest cohort of Chinese patients with cryoglobulinemia. We found that renal involvement and HBV infection might be more common in Chinese patients with cryoglobulinemia.

Anaplastic large cell lymphoma in a patient with MAGIC syndrome: a case and review of the literature.

Mouth and genital ulcer with inflamed cartilage syndrome (MAGIC syndrome) is a rare autoinflammatory disorder with unknown etiology. Except for the common clinical manifestations mimicking Behçet's disease and relapsing polychondritis, some other clinical entities are occasionally observed. In this report, we present a case in which a patient developed anaplastic large cell lymphoma 1 year after the diagnosis of MAGIC syndrome. Additionally, we review the clinical manifestations, management, and prognosis of MAGIC syndrome.

Risk and prognosis factors for systemic sclerosis with lung cancer: A single-centre case-control study in China.

OBJECTIVE: To analyse clinical characteristics, risk and prognosis factors for systemic sclerosis (SSc) patients with lung cancer. METHODS: SSc patients with lung cancer admitted to Peking Union Medical College Hospital from February 1992 to December 2018 were included. Age and sex-matched controls were selected from a pool of SSc patients without lung cancer during the same period. Conditional logistic regression and Cox proportional-hazard regression were used to identify risk factors and prognosis factors. The Kaplan-Meier method was used to draw the survival curve and calculate median survival. RESULTS: Nineteen SSc patients with lung cancer and 76 controls were included. The mean age at lung cancer diagnosis was 54.4 ± 10.2 years. In all 19 cases the lung cancer had been diagnosed after SSc and the median interval between SSc onset and lung cancer onset was 10.5 years (range 2.0-36.2 years). Among SSc patients with lung cancer, the median follow-up time and median survival were 2.6 years and 1.4 years, respectively. In the sex and age-matched conditional logistic multivariable regression analysis, family history of malignancy (OR 4.930, 95%CI 1.926-12.619, P = .001), ILD (OR 7.701, 95%CI 1.009-58.767, P = .049) were independent risk factors for lung cancer among SSc patients, and considering sex and age of SSc onset, SSc patients with more advanced staging of lung cancer (HR 3.190, 95%CI 1.127-6.126, P = .06) had poorer prognosis. CONCLUSION: Lung cancer is not uncommon in SSc patients, especially those with family histories of malignancy or ILD. Early detection of lung cancer is of vital importance for better prognosis.

Malignancy and IgG4-related disease: the incidence, related factors and prognosis from a prospective cohort study in China.

This prospective cohort study aims to investigate the incidence, related factors and prognosis of IgG4-related disease (IgG4-RD) with malignancies in the Chinese cohort. We prospectively analyzed the IgG4-RD patients recruited in Peking Union Medical College Hospital from January 2011 to August 2018 and identified patients diagnosed with IgG4-RD complicating malignancies. Data regarding demographics, clinical features, treatment and prognosis of IgG4-RD patients complicating malignancies were collected and compared to those of age- and sex-matched controls. Among the 587 Chinese patients with IgG4-RD, 17 malignancies were identified. Ten of them developed malignancy after the diagnosis of IgG4-RD, given a standard incidence ratio (SIR) of 2.78 (95%CI 1.33-5.12). Multivariate logistic analysis indicated that autoimmune pancreatitis (OR = 6.230, 95%CI 1.559-24.907, p = 0.010) was positively associated with malignancy, whereas eosinophilia (OR = 0.094, 95%CI 0.010-0.883, p = 0.039) was negatively related with malignancies. During a median follow-up period of 61.4 ± 26.4 months, all patients with IgG4-RD and malignancies survived. We conclude that an increased incidence of malignancy was found in Chinese IgG4-RD cohort. Autoimmune pancreatitis is a potential risk factor, whereas eosinophilia is a possible protective factor for complicating malignancies.

Clinical characteristics of cytomegalovirus gastritis: A retrospective study from a tertiary medical center.

Cytomegalovirus (CMV) gastritis is a rare opportunistic infection with diverse clinical manifestations. Our study aimed to investigate the clinical features of Chinese patients with CMV gastritis.Six inpatients diagnosed with CMV gastritis were retrospectively enrolled, based on the finding of inclusion bodies in routine hematoxylin and eosin staining or positive anti-CMV monoclonal antibodies under immunohistochemistry in the gastric biopsy. Data, including demographics, diagnostic measurements, and medications, were collected.Abdominal pain was the most frequently reported symptom, occurring in 4 patients. Five patients were immunocompromised with associated underlying diseases, and 3 patients had decreased leukocyte differentiation antigen 4 positive (CD4) T lymphocyte counts. Only 3 patients had either positive cytomegalovirus (CMV)-immunoglobulin (Ig) M or increased copies of CMV-DNA peripherally. All patients had gastric lesions in the antrum of the stomach, including ulcers or erosions observed by gastroscopy. All patients received ganciclovir by intravenous injection (IV) as the first line anti-CMV therapy, and attained complete (4) or partial remission (2) during the follow-up.CMV gastritis should be taken into consideration in patients with immunocompromised status who have abdominal pain, nausea, or vomiting. Gastroscopy and necessary biopsy are the major diagnostic methods for CMV gastritis. Early diagnosis leads to a better prognosis for these patients.

Cardiac Involvement in Eosinophilic Granulomatosis With Polyangiitis: A Retrospective Study in the Chinese Population.

Introduction: Cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA) is associated with a poor prognosis and high mortality; however, few studies about cardiac involvement in EGPA in the Chinese population are available. We conducted this study to determine the clinical characteristics and overall outcomes of Chinese EGPA patients with cardiac involvement. Materials and Methods: We retrospectively collected the clinical data of 83 patients diagnosed with EGPA and analyzed the differences between the patients with and without cardiac involvement. Results: The prevalence of cardiac involvement in EGPA in this cohort was 27.7%. Compared with those without cardiac involvement, EGPA patients with cardiac involvement tended to have a younger age at onset (mean ± SD: 38.4 ± 10.5 vs. 42.1 ± 15.9 years, respectively, p = 0.039), higher eosinophil count (median [IQR]: 5810 [4020-11090] vs. 2880 [1530-6570] n/µL, respectively, p = 0.004), higher disease activity assessed using the Birmingham vasculitis activity score (BVAS) (median [IQR]: 20 [16-28] vs. 15 [12-18], respectively, p = 0.001), and poorer prognosis (Five Factor Score [FFS] ≥ 1: 100% vs. 38.3%, respectively, p = 0.001). In the cardiac involvement group, 43.5% of patients were asymptomatic, but cardiac abnormalities could be detected by cardiac examinations. With appropriate treatment, the overall outcomes of EGPA patients with cardiac involvement in our cohort were good, with only 3 (13.0%) patients dying in the acute phase and no patients dying during follow-up. Conclusions: Cardiac involvement in EGPA was associated with a younger age at onset, higher eosinophil count, higher disease activity, and a poorer prognosis. Comprehensive cardiac examinations and appropriate treatment are essential to improve the prognosis of those with cardiac involvement.

Pituitary Involvement in Granulomatosis with Polyangiitis: A Retrospective Analysis in a Single Chinese Hospital and a Literature Review.

PURPOSE: Granulomatosis with polyangiitis (GPA) is an anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis that can involve virtually many organs, including the pituitary. Pituitary involvement in GPA is rare, with only case reports or small case series published previously. METHODS: We used the electronic medical record system in our hospital to identify four patients of pituitary involvement in GPA. We summarized the clinical characteristics, radiographic findings, treatments, and clinical outcomes of the four patients. We further performed a systematic literature review of 66 GPA cases with pituitary involvement that were published on the PubMed database. RESULTS: The four women in our report were between 57 and 73 years of age. All patients had pituitary abnormalities on radiology; three developed diabetes insipidus (DI). All patients had multisystem involvement. After treatment with glucocorticoids and cyclophosphamide (CYC), all patients showed clinical improvement but pituitary function did not resume. Literature review identified 66 additional patients with pituitary involvement in GPA; diabetes insipidus (57/66, 86.4%) and hypogonadism (34/66, 51.5%) were the most frequent pituitary disorders, and the most frequent imaging lesion was an enlarged pituitary (25/64, 39.1%). After treatment with corticosteroids and/or immunosuppressive agents, most patients (45/66, 68.2%) developed remission from systemic disease, 13 patients (13/57, 22.8%) showed remission of DI, and 8 patients (8/46, 17.4%) showed remission of hormone deficiencies. CONCLUSIONS: GPA should be carefully considered as a potential cause of pituitary dysfunction (PD), especially when multisystem dysfunction exists. Conventional treatment with corticosteroids and/or immunosuppressive agents improves systemic symptoms, but pituitary disorders persisted in most patients.

[Clinical characteristics of patients with relapsing polychodritis].

OBJECTIVE: To analyze the clinical characteristics of relapsing polychodritis (RP). METHODS: Clinical features and laboratory data of 131 patients with RP were analyzed retrospectively. RESULTS: The average age at onset was (44 ± 14) years (ranging from 9 to 76). Male to female ratio was 1.22:1. The most common onset symptom was respiratory system symptom (61.8%), followed by auricle lesions (16.0%), articular cartilage (15.3%), ocular region (7.6%), internal ear system (5.3%), nasal cartilages (4.6%), skin (2.3%) and nervous system (0.8%). During the whole course of the disease, 81.7% of the patients endured a respiratory system involvement; 77.9% and 56.5% of the patients suffered joint symptoms and nasal cartilages lesions, respectively; the involvement of auricle lesions, ocular region and internal ear system were 54.2%, 35.1% and 33.6%. Comparatively, the occurrence rates of skin lesion (10.7%), neurologic disorder (9.2%) and kidney system lesions (1.5%) were low. Analyzing of the laboratory examination data showed that 38.2% and 43.5% of the patients had an increase in white blood count (WBC) and platelet, 86.3% and 82.4% patients showed raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) values. CONCLUSIONS: RP is a multi-system involvement disease, which has complicated clinical manifestations. Respiratory tract is the most common site involved, and respiratory system involvement is a prediction of unfavourable prognosis.