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• W2039631566 abstract "We read with interest the article by Wulffraat et al. 1Wulffraat NM de Graeff-Meeder ER Rijkers GT van der Laag H Kuis W Prevalence of circulating immune complexes in patients with cystic fibrosis and arthritis.J P EDIATR. 1994; 125: 374-378Scopus (17) Google Scholar about cystic fibrosis (CF) and arthritis. The authors affirm that episodic arthritis in patients with CF resembles reactive arthritis and the arthritis of Reiter syndrome, disorders associated with the presence of HLA-B27. This statement, however, is not completely correct because reactive arthritis and Reiter syndrome, usually much more frequent in patients in whom HLA-B27 antigen is present, can also develop in patients without HLA-B27 antigen, even in the absence of its cross-reactive antigens, such as HLA-B7, HLA-B22, and HLA-B40. 2Ford DK Reiter's syndrome: reactive arthritis.in: Arthritis and allied conditions. Lea & Febiger, Philadelphia1989: 944-953Google Scholar Wulffraat et al. also discuss the character of CF arthritis, underline the limited clearance capacity of the reticuloendothelial system in this situation, and describe the results of sputum cultures of patients, but they do not stress the importance of Pseudomonas aeruginosa colonization in the pathogenesis of arthritis or vasculitic lesions associated with CF episodic arthritis. Sixteen patients with purpuric rash and five of eight patients with CF arthritis described by Schidlow et al. 3Schidlow DV Goldsmith DP Palmer J Huang NN. Arthritis in cystic fibrosis.Arch Dis Child. 1984; 59: 377-379Crossref PubMed Scopus (44) Google Scholar, 4Schidlow DV Panitch HB Zaeri N Zenel J Alpert BE. Purpuric rashes in cystic fibrosis.Am J Dis Child. 1989; 143: 1030-1032PubMed Google Scholar had Pseudomonas species in their respiratory secretions. In our center, three of 125 patients (two male subjects and one female subject) have episodic arthritis. The age at onset was the second decade of life. All these patients have liver involvement, have chronical P. aeruginosa colonization, and have high levels of anti- P. aeruginosa antibodies. Arthritis is pauciarticular and asymmetric; the most commonly affected joints are the knees, ankles, and proximal interphalangeals, and in two cases purpuric rash is present on the skin of the lower extremities. Laboratory investigations reveal an increased erythrocyte sedimentation rate and elevated levels of C-reactive protein and immunoglobulins. Results of tests to detect antinuclear antibody and rheumatoid factor are negative. The third and fourth components of complement, total hemolytic complement, and uric acid values are all within normal range. Enzyme-linked immunosorbent assay to detect circulating immune complexes binding the C1q and C3d subunits of complement, detectable in 20.3% of all our patients with CF, indicates the presence of these substances in two patients with CF arthritis. No association with HLA type has been shown. Recurrence of acute arthritis had been described by the patients before antibiotic treatment was administered. The duration of episodes varied from 2 to 7 days with recurrence at intervals of several weeks. Episodic arthritis has been nondestructive, without progression to erosive arthritis. Our patients are usually treated with nonsteroidal antiinflammatory drugs, but in some episodes arthritis remitted spontaneously. The clinical pattern and the frequent association with purpuric rash may be related to circulating immune complex arthritis. In our experience, 5Taccetti G Campana S Ferrari R. Cystic fibrosis and acute episodic arthritis: our experience.Clin Exp Rheumatol. 1994; 12: S-134Google Scholar routine rheumatologic studies fail to reveal any explanation for the nature of these attacks, but continuous antigenic stimulation produced by the persistence of P. aeruginosa may be responsible for im mune stimulation, production of antibodies against P. aerugi nosa and immune complexes. Tissue deposition of circulating immune complexes containing P. aeruginosa antigen and a malfunction of clearance by the mononuclear phagocyte system in the damaged liver could be responsible for acute episodic CF arthritis. 9/35/63324" @default.
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• W2039631566 date "1995-05-01" @default.
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• W2039631566 title "Cystic fibrosis and episodic arthritis" @default.
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