O. Kaye

Effective treatment of Jo-1-associated polymyositis with T-cell-depleted autologous peripheral blood stem cell transplantation

A patient with Jo‐1 antibody‐associated polymyositis (Jo‐1 PM) had a Karnofsky score of 40% and severe muscle, liver and lung damage that was refractory to standard therapy. The female patient received an autologous T‐cell‐depleted haematopoietic stem cell transplant (HSCT) after myeloablative conditioning. The transplant procedure was complicated by severe adult respiratory distress syndrome (ARDS) and adenovirus‐associated haemorrhagic cystitis as well as cytomegalovirus (CMV) reactivation. The patients creatinine phosphokinase (CPK) and alanine transaminase (ALT) values were normal on day 21. The patients strength has improved remarkably and her dyspnoea is subjectively improved. At 15 months after the transplant, the patient was well with a Karnofsky score of 80% and had been off any therapy, including steroids, for 14 months.

Increased synovial fluid levels of soluble CD23 are associated with an erosive status in rheumatoid arthritis (RA)

Synovial fluid (SF) levels of soluble CD23 (sCD23) were determined in 96 patients presenting with an inflammatory knee effusion (73 with RA and 23 with reactive arthritis (ReA) serving as a control inflammatory non‐erosive group) and were correlated with the degree of joint destruction, with local immune parameters (IL‐1β, IL‐3, IL‐4, IL‐6, IL‐8, IL‐10, IL‐12 and sCD25) and with serum markers of inflammation, C‐reactive protein and erythrocyte sedimentation rate. RA patients, classified as erosive or not according to Larsen’s grade, were separated as follows: (i) 13 patients with non‐erosive RA; (ii) 16 RA patients with erosions in hands but not in knees, matched for disease duration with the first group; (iii) 44 RA patients with hand and knee erosions, matched with the second group for rheumatoid factor positivity but of longer disease duration. SF sCD23 levels were significantly increased in both erosive RA groups compared with non‐erosive diseases, whether RA or ReA (P < 0·05), whose SF levels were not different. SF IL‐10 showed a similar profile to that of SF sCD23 and was the only other parameter characteristic of erosive RA, but no direct correlation was found between the two. SF sCD23 was significantly correlated with IL‐12 (r = 0·65, P = 0·0001) and sCD25 (r = 0·39, P = 0·0019) exclusively in the two erosive RA populations. In conclusion, these data showing that increased levels of sCD23 are not only found in the SF of erosive joints but also in knee SF of patients with erosive RA but without knee x‐ray‐diagnosed erosions suggest that this parameter might be of predictive value for joint destruction. Longitudinal studies are however needed to confirm its potential clinical interest.

Néphropathie tubulo-interstitielle aigue avec uvéite: à propos d'un cas

We report another case of acute interstitial nephritis with uveitis (TINU syndrome) in a 35-year-old woman. About thirty cases were described since the first ones 20 years ago. We discuss the assessment needed to reach the diagnosis. The evolution is unusually favourable with steroid therapy.

Sarcoidosis: recognition and treatment guidelines.

Sarcoidosis is a systemic disorder of unknown aetiology characterised by noncaseating granulomas leading principally to bilateral hilar lymphadenopathies, pulmonary infiltration and skin and eye lesions. Sarcoidosis may involve other organs, including peripheral lymph nodes, liver, spleen, nervous and musculoskeletal systems, heart, ear, nose and kidney. Although the clinical involvement of liver and heart is relatively uncommon, hepatic and cardiac granulomas are present at autopsy in about 70 to 80% and 25 to 50%, respectively, of patients with this disease. The diagnosis of sarcoidosis includes compatible clinical and/or radiological presentations and histological evidence of noninfectious and noncaseating epitheloid cell granulomas in the absence of other identifiable agents responsible for such histological lesions. Disease course is variable and usually characterised by frequent remissions, but it may become progressive and chronic in a small percentage of patients. The optimal treatment of sarcoidosis remains poorly defined. In patients with progressive pulmonary dysfunction as well as in those with severe extrapulmonary localisations, systemic corticosteroids usually represent the first approach, limited by long term toxicity and frequent relapses after treatment interruption. In the presence of refractory or corticosteroid-dependent forms of the disease, antimalarial drugs or low dosage methotrexate may be used with prolonged benefit. The indications for immunosuppressive agents such as azathioprine, chlorambucil, cyclophosphamide and cyclosporin are uncommon and limited because of potentially serious adverse effects and lack of information on their long term efficacy. In the case of ocular and limited cutaneous manifestations, local corticosteroid therapy may be useful.