Clinical Studies Of Early Diagnosis In Adult Onset Still's Disease And Spondyloarthritis

Part I : Clinical Study of Adult Onset Still's Disease Objective1. By retrospective studying of in-patients with adult onset still's disease (AOSD), we expect it can help early diagnosis of AOSD, and reduce missed diagnosis and misdiagnosis. 2. To evaluate the clinical value of serum ferritin(SF) and glycosylated ferritin(GF) in the diagnosis of AOSD.Methods246 cases of AOSD patients, admitted to our department from January 1993 to March 2007, were retrospectively investigated. All the cases satisfied the Yamaguchi's criteria. Clinical data of 65 patients with fever of unknown origin (FUO) were collected, and 5ml blood from every patient and 11 healthy controls were collected to measure the levels of SF and GF. According to the final diagnosis, patients were divided into AOSD group and non-AOSD group which were further divided into subgroups including infection, other rheumatic diseases, tumor and others. SF level was determined by electrochemiluminescence immunoassay. The percentage of GF in whole SF was analyzed by concanavalin-A binding method.ResultsA. Retrospective clinical study of 246 patients with AOSD1. General situation: The mean age at onset was 33.06±13.47 years and the female/male ratio was 2.1.2. Clinical features: The first manifestation in 81.3% of AOSD patients was fever, and 76.8% of patients had fever, rash and arthralgia simutanously. All patients had fever during their course. Remittent fever was the commonest and appeared in 73.6% of patients. Rash appeared in 91.1%, mainly macule and maculopapule, and was associated with fever in 65.6% of patients. Arthralgia appeared in 85.8%, and 78.2% of them were assoicated with fever. Sore throat, myalgia, lymphadenectasis, splenohepatomegalia, hydrohymenitis and weight loss were not uncommon.3. Laboratory examination: The percentage of leucocytosis, neutrophil ≥ 80% and anaemia were 67.4%, 63.7% and 49.8% respectively. ESR, CRP and SF levels were elevated in active stage, and their frequences was 94.2%, 92.9% and 56.5% respectively. ALT/AST, LDH or γ-GT/ALP were increased in almost half of patients and Alb was decreased in 63.8%. ANA and RF were negative in over 90% patients. Radiographic examination of joints were performed in 72 cases and 13.9% of patients had joint space narrowing, erosion or osteoporosis. Wrist was the most involved joint. Infective change was the commonest finding in bone marrow examination and its frequence was 51.3%. 78.9% of pathological findings of lymph node were reactive hyperplasia. Biopsy of spleen, liver or skin were performed in a few patients and most of them were non-specific pathological changes.4. The WBC count changes: After treatment with moderate to high dose of corticosteroid within 20 days, WBC count returned to normal in a minority of patients, while in half of patients WBC was higher than that before the therapy. But after 20 days, WBC in the majority of patients returned to normal, and only in a few patients it was higher than that before the treatment.B. The diagnostic value of SF and GF in AOSD1. Clinical data: 65 patients of FUO (29 cases of AOSD, 36 others) were invovled, the M : F ratio was 1 : 1.83 and the mean age was 38.48±16.38 years. In comparison of the two groups, we found that the clinical manifestation and abnormal examination seen in AOSD were also common in other disease with fever only with a lower frequency. While the remittent fever, characteristic rash, sore throat, myalgia and lymphadenectasis were more common in AOSD patients (PO.05).2. SF result: The SF level in AOSD group (8370.57±9370.45ng/ml) was much higher than that in non-AOSD group (1897.16±3328.74ng/ml) and the group of other rheumatic disease (P<0.01). The SF level was significantly different between AOSD group and the group of merely infective disease (P<0.05), but no statistical significance was found between AOSD group and tumor group (P>0.05). SF level in each groups of different diseases was much higher than that in the normal control group (P>0.05).3. GF result: The GF in AOSD group (20.58± 14.06%) was much lower than that in non-AOSD group (51.92±23.46%, P<0.01), as well as group of other rheumatic diseases(51.99±21.37%, P<0.01) or group of merely infective diseases(72.64±9.98%, P<0.01). The GF level in AOSD group was much lower than that in tumor group(41.10±24.05%, P<0.05)., GF lever in every group except the infective diseases was much lower than that in normal control group (P>0.05).4. The SF and GF in stable AOSD: The mean SF in 4 stable patients was 321.4 ng/ml, which was lower than that active AOSD, but no statistical significance was found (P>0.05). The mean GF was 42.03%, which was much higher than that in active AOSD (P<0.05).5. Sensitivity and specificity of SF and GF in diagnosis of AOSD: If the cutoff value of SF is 1500ng/mI, its sensitivity is 74.07% and specificity is 77.78%. If the cutoff value of GF is 20%, its sensitivity is 62.96% and specificityis 88.89%. When the two values are analyzed together, the sensitivity is 59.26% and the specificity is 94.44%.Conclusions1. Fever, rash and arthralgia are the most important symptoms. Pharyngalgia, myalgia, lymphadenectasis, splenohepatomegalia, hydrohymenitis and weight loss are common.2. Serum SF level in active AOSD is obvious higher than that in other febrile diseases.3. The proportion of GF in active AOSD is obvious lower than that in other febrile diseases, and may come back to normal with the disease being gradually controlled.4. Serum SF and GF may be helpful for the diagnosis of AOSD.Part II: The Clinical Value of MRI of Sacroiliac Joints in Early Diagnosis of SpondyloarthritisObjectiveTo study the clinical value of magnetic resonance imaging (MRI) of sacroiliac joints (SIJs) in the early diagnosis of spondyloarthritis.MethodsPatients with chronic back pain at out-patient clinic of rheumatology department were collected, and their MRI of SIJs, X-rays of SIJs and lumbar spine, HLA-B27, CRP as well as scoring with Amor criteria were performed. Final diagnosis was made based on the complete data, and the patients were divided into two groups according to the nature of back pain (inflammatory back pain (IBP) of A group and mechanical back pain of B group). Patients with IBP was subgrouped by the, manifestation of MRI of SIJs.Results1. 39 patients with chronic back pain were included. 7 patients with mechanical back pain hardly met Amor criteria, with the highest score as 2 and MRI of SIJs all normal.2. Most of the X-rays and computed tomography (CT) of SIJs in the 30 patients with IBP were normal or only have doubtful lesions. While MRI showed that 22 patients had acute active inflammatory lesions of SIJs and no abnormality was found in other 8 patients. In a few patients with slight radiographic abnormaligy in SIJs. MRI can not only confirm the lesions but also tell the extent of the inflammation.3. Two patients with predominantly peripheral symptoms have no back pain and buttock pain, and their scores of Amor criteria are both 6. MR1 showed that there were obvious acute inflammatory lesions in SIJs.4. No significant difference was found between A and B groups in the following items: morning stiffness, asymmetric peripheral arthritis, enthesitis, dactyl it is, uveitis, positive HLA-B27, acute inflammatory lesions in MRI of the spine and light lesions in CT of SIJs. While more patients in the A group (54.5%) had scores of Amor criteria over 6 than those in the B group (12.5%, p<0.05).5. In patients with IBP and Amor score > 6, the diagnosis of SpA is supported with lesions found in MRI of SIJs, while possibility of axial SpA is small without lesion in MRI and the patients need follow-up observation. In patients with IBP and Amor score < 6, MRI of SIJs should be performed when the diagnosis of axial SpA cannot be excluded, and the diagnosis is supported if there are lesions in MRI of SIJs.Conclusions1. Patients with mechanical back pain seldom have the terms of Amor criteria, and findings in MRI of SIJs are all normal.2. MRI of SIJs can find early sacroiliitis, which is valuable for early diagnosis of SpA and is important for differential diagnosis of patients with IBP.3. SpA patients with predominant peripheral involvement may have sacroiliitis.