Prompt remission of severe SLE with only three doses of rituximab infusion and low dose steroid: the first case report from Indonesia.

A 31-year-old Chinese lady presented with severe SLE with nephrotic syndrome, anemia, leucopenia and thrombocytopenia, skin lesions, and joint inflammation after failing previous standard therapy. After treatment with three infusions of rituximab she showed immediate improvements regarding clinical and laboratory parameters. She received no cytotoxic drugs and remained well for at least 7 months, despite stopping prednisolone.

Spondyloarthropathies in eastern Asia.

The association between HLA-B27 and the spondyloarthropathies (SpAs) is so strong that it is supposed that the HLA-B27 molecule plays a pathogenetic role. In whites and Indonesians, the frequency of HLA-B27 is about 10%; in Chinese it is about 8%; and in Japanese it is only about 1%. The prevalence of SpA in the Chinese is at least 0.2%, but in native Indonesians, Philippinos, and Malaysians, SpA is rarely seen. Twenty-three subtypes (B*2701-B*2723) have been distinguished. These subtypes are not equally distributed over the world. In most countries the distribution of the subtypes among HLA-B27 SpA patients is the same as that among the normal HLA-B27-positive population. In China, the subtype B*2704 is frequent and the prevalence of SpA is high. Native Indonesians, however, mostly have subtype B*2706, and SpA is rarely seen in this population. It was shown that B*2706, probably like B*2709 in Sardinia, is not associated with SpA. The difference between the SpA-associated and non-SpA-associated subtypes is limited to only two amino acid residues (114 and 116) at the bottom of the peptide-binding groove of HLA-B27. This small difference between health and disease rewards studies for different peptide-binding capacities and may help us characterize the peptides that are involved in the pathogenesis of SpA. The differences in disease associations in these countries also have clinical implications. In Southeast Asia, HLA-B27 typing without subtyping has less clinical usefulness than in parts of the world where B*2706 is rarely seen. When native Indonesians, Malaysians, or Philippinos are suspected of having ankylosing spondylitis or a related SpA, it is worth asking if they had white or Chinese ancestors. If native HLA-B27-positive Indonesians (with subtypes other than B*2706) develop SpA, the clinical features are not different from those in other parts of the world. In the Chinese population on the mainland and in Taiwan, juvenile SpA is frequently seen. The onset is often a peripheral arthritis or enthesitis.

Evidence that HLA-B*2706 is not protective against spondyloarthropathy.

OBJECTIVE: Studies in Southeast Asia showed that HLA-B*2704 is positively associated with spondyloarthropathy (SpA), while B*2706 does not occur in such patients. In view of the absence of an association between B*2706 and SpA it was suggested that B*2706 protects against the disease, while it is supposed that B*2704 presents pathogenetic peptides. We studied families in which both B*2704 and B*2706 occurred to see whether in B*2704/B*2706 heterozygotes the effect of one of the subtypes shows a preponderance over the other. METHODS: Two families of mixed Chinese/Indonesian origin were studied. HLA-B27 subtyping was performed by polymerase chain reaction in combination with sequence specific oligonucleotide probes. RESULTS: In one family, members with B*2704, B*2706, or both occurred. In the other family B*2704, B*2706, and B*2708 were present. In both families SpA was seen only in B*2704 positive members, while the B*2706 and B*2708 positive members were healthy, except some B*2704/B*2706 or B*2704/B2708 heterozygotes. CONCLUSION: The pathogenic influence of B*2704 is thus dominant over the supposed protective influence of B*2706. It is probable that B*2704 can present pathogenetic peptides, while a protective influence of B*2706 does not exist. B*2708, which was until now described in only a few cases, behaved in this study as B*2706 and is probably not associated with SpA.

Clinical features of spondyloarthropathy in Chinese and native Indonesians.

The prevalence of spondyloarthropathy (SpA) in Chinese Indonesians is much higher than in native Indonesians. This is due to HLA-B27 subtype differences. In the present study we re-examined the clinical features of SpA in Indonesians to see whether, besides the HLA-B27 subtype differences, other factors affect the frequency of SpA. Seventy two patients with SpA were re-examined. The patients came from two clinics for rheumatic diseases. The overall entry ratio of Chinese to native Indonesians was 1:2. Ankylosing spondylitis (AS) was more frequent among the Chinese (n = 32, 94% B27 positive) than among the native Indonesians (n = 5, 40% B27 positive). HLA-B27 subtyping was performed on 22 of the 37 HLA-B27-positive AS patients. Twenty Chinese were positive for B*2704 and two native Indonesians were B*2705 positive. The clinical features of AS and reactive arthritis (ReA) showed no differences between the two populations and were similar to the clinical descriptions in other parts of the world. In conclusion, it can be stated that in spite of HLA-B27 subtype differences the clinical features of SpA in Chinese and native Indonesians are fully comparable.

HLA-B27 subtypes positively and negatively associated with spondyloarthropathy.

OBJECTIVE: Eleven subtypes of HLA-B27 have been identified. If some of these subtypes had a stronger association with spondyloarthropathy (SpA) than others, this might tell us which peptides are of pathogenetic importance. A subtype preponderance has not been proved in Caucasians or in Asian Indians. Our objective was to determine whether some subtypes are positively or negatively associated with SpA in Indonesia. METHODS: Cells of 34 HLA-B27 positive patients with SpA (fulfilling the European Spondylarthropathy Study Group criteria) and 26 HLA-B27 positive controls, all living in Java, Indonesia, were sampled. Patients and controls were divided according to their presumed ethnic origin. HLA-B27 subtyping (B*2701-09) was performed by polymerase chain reaction in combination with sequence specific oligonucleotide probes to analyze polymorphism in exons 2 and 3 of HLA-B27. RESULTS: HLA-B*2701, *2702, *2703, *2708, and *2709 were found in neither group. HLA-B*2704 was found in 23/34 (68%) of the patients and in only 4/26 (15%) of the controls (p < 0.01). HLA-B*2706 was found in none of the 34 patients, but in 21/26 (81%) of the controls (p < 0.01). One drawback of the study was that most patients were of Chinese descent and most controls were native Javanese. Nevertheless, the absence of SpA among HLA-B*2706 positive individuals is noteworthy. CONCLUSION: HLA-B*2704 is positively associated with SpA (RR = 11.5), while *2706 is negatively associated with this disease (RR < 0.007). The results confirm the findings of Lopez-Larrea, et al in Thailand.

Higher relative risk of spondyloarthropathies among B27 positive Indonesian Chinese than native Indonesians.

We assessed the prevalence of HLA-B27 among ethnic Indonesian and Indonesian Chinese patients with ankylosing spondylitis (AS) or related spondyloarthropathies, and also among healthy controls. HLA-B27 was found in 23 (62.2%) of 37 Chinese patients and 4% of 176 Chinese healthy controls (p < 0.001). In contrasts only 2 (8.3%) of 24 native Indonesian patients and 13 (9%) of 145 healthy controls were HLA-B27+, indicating the lack of association of HLA-B27 with spondyloarthropathies in native Indonesians. These findings also suggest that HLA-B27 Chinese Indonesians carry a greater relative risk of developing AS and related spondyloarthropathies than native Indonesians, although the prevalence of HLA-B27 in the general population is more than 2 times higher in native Indonesians.

Prognostic factors in juvenile chronic arthritis.

We evaluated 96 patients (50 males, 46 females) with juvenile chronic arthritis (JCA) for various prognostic factors in an adult rheumatology clinic. Although the onset of JCA occurred before the age of 15 in all cases, the majority had a juvenile or late onset of disease. The mean duration of disease was 14 years. Twenty-eight % had a monoarticular onset, 26% a pauciarticular, 28% a polyarticular and 14% a spondylarthropathic onset. HLA-B27 was positive in 52% of the cases, 35 males and 12 females, and HLA-DW4 was present in 10%; 11.5% were ANA positive and 4% were found rheumatoid factor positive (latex greater than 1/128). Patients were classified in functional classes, using a slight modification of Steinbrocker's criteria. Patients who underwent major orthopaedic surgery of the hip or knee were classified in functional class IV, although they actually showed better function. Twenty-seven % had a functional class I, 45% class II and 24% class III-IV at the latest evaluation. In the group with poor prognosis (functional class III and IV) there were significantly more cases with a persistently high erythrocyte sedimentation rate; polyarticular involvement at onset and at the time of their last evaluation; and a family history of rheumatic diseases. There were significantly more females in the poor prognosis group. The presence of HLA-B27 and an ANA positive test were not significantly different in the functional class groups. HLA-B27 did not predict the development of typical ankylosing spondylitis but was associated with pauciarticular peripheral arthritis with or without mild spondylitis.

Double-blind trial comparing bufexamac infiltrations with triamcinolone acetonide infiltrations in patients with periarthritis of the shoulder.

A double-blind trial was carried out in 40 patients with an acute episode of periarthritis of the shoulder to compare the effectiveness and toleration of local infiltrations of bufexamac and triamcinolone acetonide. Patients received 1 or 2 local infiltrations with either 20 mg bufexamac or 40 mg triamcinolone acetonide. The results were evaluated by objective and subjective assessments of pain, loss of function, mobility, and by doctors' and patients' opinions of relief. There was a significant, comparable reduction in pain and discomfort in both groups after the first injection. Fourteen patients in each group required a second injection, with slightly more improvement in those receiving bufexamac. Local intolerance of the injections was noted in approximately 25% to 30% of the patients.