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REITER'S SYNDROME
567 which is characteristic is the lack of uppertobe blood diversion, despite Kerley B lines. Thus, on chnical grounds the diagnosis can be strongly suspected, although final proof still depends on seeing the characteristic histological abnormalities in the lung. The prognosis of pulmonary veno-occlusive disease is l1lS1na[5 and conservative treatment with anticoagulants,6 steroids,’ digoxin, and diuretics has made little impact. Only one patient, reported by Sanderson et al.,8 has survived for more than two years. In this patient the diagnosis was made from a lung biopsy specimen. The patient seems to have improved strikingly after treatment with azathioprine, but she differed from previous cases because of associated Raynaud’s phenomenon, arthritis, vasculitic ulcers, and high IgG and IgM levels. This response to an immunosuppressive drug indicates that the damage to the small pulmonary veins is not caused by a specific agent but that various injuries may result in the same histological appearance. It is unknown why the small pulmonary veins are the site of the disease in preference to the- alveoli or arterioles. The consequence of this small-vein disease, however, is a condition which is readily diagnosed clinically. Wider recognition and earlier diagnosis will help to clarify the xtioiogy and speed progress towards an effective treat-
radiograph
ment.
REITER’S SYNDROME ITis 63 years since Reiter described his syndrome of initial diarrhoea, urethritis, conjunctivitis, and arthritis in males, but with the passage of the years the definition seems ever less adequate. At a symposium held last year in Lausanne and now published,’ D. A. Brewerton pointed out that Reiter’s syndrome is always incomplete; and G. W. Csonka had to see 410 patients to appreciate the complete picture of the disease. Possibly the variations in clinical features are due to genetic factors-with the actual environmental trigger having little influence on the way in which each patient responds. The interrelated clinical features which are customarily classed within the entity "Reiter’s syndrome" now include urethritis (presumably sexually acquired), balanitis, ker-
atoderma, stomatitis, conjunctivitis, uveitis, peripheral asymmetrical arthritis, atypical spondylitis, and occa-
sionally aortitis and lesions of the central nervous system. Beyond this are still other conditions which may be associated with Reiter’s syndrome, such as bacillary dysentery, ulcerative colitis, Crohn’s disease, intestinal yersinia infection, and psoriasis. The gene B 27 is present in 7% of the population in Britain and in about 65% of patients with Reiter’s syndrome with peripheral arthritis alone; but if features such as sacroiliitis, uveitis, or balanitis are also present - each of these individually associated with B 27-then the prevalence of B 27 is almost 100%. Crucially, idio6 Brown CH, Harrison CV. Pulmonary veno-occlusive disease. Lancet 1966; ii 61-66. 7. Weisser K, Wyier F, Gloor F. Pulmonary veno-occlusive disease. Arch Dis Child 1967; 42: 322-27. 8 Sanderson JE, Spiro SG, Hendry AT, Turner-Warwick M. A case of pulmonary veno-occlusive disease responding to treatment with azathioprine. Thorax 1977; 32: 140-48. 1. Symposium on Reiter’s syndrome. Ann Rheum Dis 1979; 38: suppl 1.
pathic ankylosing spondylitis carries a 95% prevalence antigen, but if the disease is associated with psoriasis, ulcerative colitis, or Crohn’s disease, the prevalence of B 27 drops to around 65%. A possible explanation is that the genes of inflammatory bowel conditions and psoriasis contribute to the development of spondylitis making the presence of B 27 less necessary. The varying clinical picture and the lower prevalence of
of B 27
B 27 when some of the associated diseases are present suggest that several genes are involved to help, hinder, or mask each other. On the clinical side both Csonka and A. Catin and colleagues reported that the long-term outlook was less favourable than indicated in some tests, with a 40% rate of late disability, predominantly due to chronic foot pain and less commonly to recurrent uveitis with loss of vision. T. Bitter and co-workers described a series of young adults with persistent yet reversible seronegative pauciarthritis in whom the prevalence of B 27 was 47.5%-i.e., somewhere between rheumatoid arthritis (RA) and Reiter’s syndrome. On American Rheumatism Association criteria this homogeneous group accounted for almost 90% of all "possible" or "probable" RA seen. Incomplete Reiter’s syndrome is not a happy description for this group, which seems to deserve a designation of its own. Csonka suggested that urethritis should always be excluded by a search for early-morning urethral pus, which may be missed after micturition. There was no consensus regarding the role of pathogens isolated initally from the genital or alimentary tract in Reiter’s syndrome. Although some years ago there was excitement after Chlamydia trachomatis had been found in the synovial fluid by Schachter,2 this seems to have been due to contamination in the laboratory. It is noteworthy that B 27 is a major antigen located on the short arm of human chromosome 6, a region which encodes immune responses. Environmental factors such as genital and alimentary bacteria may interact with immune reactions modulated by sex-dependent factors. Clearly our present limited knowledge indicates new directions for research. The finding of the B 27 association has raised many questions and we have reached the position where even a clear definition eludes us: as one speaker in Lausanne put it, "We all know what we are talking about but cannot define it".
MORALE AND COMMITMENT THE Government’s latest reactions in the discussions about N.H.S. consultant contracts (see p. 576) arise from a wish to improve "the morale of consultants in the N.H.S. and their commitment to it" (in Mr Jenkin’s words). The morale of some will surely be fortified by the proposed changes, but many whole-timers, especially those for whom private practice is less appealing or less available, may not feel all that uplifted. Their commitment to the N.H.S. has been and is total and these tinkerings with their contract can have little attraction for those who believe that the use of private practice as a morale booster is incompatible with the strengthening of commitment to the N.H.S. or with raising its standards. 2. Schachter
patients
J, Barnes MG, Jones JP. Isolation of Bedsoniæ from the joints of with Reiter’s syndrome. Proc Soc Exp Biol 1966; 122: 283-85.
radiograph
ment.
REITER’S SYNDROME ITis 63 years since Reiter described his syndrome of initial diarrhoea, urethritis, conjunctivitis, and arthritis in males, but with the passage of the years the definition seems ever less adequate. At a symposium held last year in Lausanne and now published,’ D. A. Brewerton pointed out that Reiter’s syndrome is always incomplete; and G. W. Csonka had to see 410 patients to appreciate the complete picture of the disease. Possibly the variations in clinical features are due to genetic factors-with the actual environmental trigger having little influence on the way in which each patient responds. The interrelated clinical features which are customarily classed within the entity "Reiter’s syndrome" now include urethritis (presumably sexually acquired), balanitis, ker-
atoderma, stomatitis, conjunctivitis, uveitis, peripheral asymmetrical arthritis, atypical spondylitis, and occa-
sionally aortitis and lesions of the central nervous system. Beyond this are still other conditions which may be associated with Reiter’s syndrome, such as bacillary dysentery, ulcerative colitis, Crohn’s disease, intestinal yersinia infection, and psoriasis. The gene B 27 is present in 7% of the population in Britain and in about 65% of patients with Reiter’s syndrome with peripheral arthritis alone; but if features such as sacroiliitis, uveitis, or balanitis are also present - each of these individually associated with B 27-then the prevalence of B 27 is almost 100%. Crucially, idio6 Brown CH, Harrison CV. Pulmonary veno-occlusive disease. Lancet 1966; ii 61-66. 7. Weisser K, Wyier F, Gloor F. Pulmonary veno-occlusive disease. Arch Dis Child 1967; 42: 322-27. 8 Sanderson JE, Spiro SG, Hendry AT, Turner-Warwick M. A case of pulmonary veno-occlusive disease responding to treatment with azathioprine. Thorax 1977; 32: 140-48. 1. Symposium on Reiter’s syndrome. Ann Rheum Dis 1979; 38: suppl 1.
pathic ankylosing spondylitis carries a 95% prevalence antigen, but if the disease is associated with psoriasis, ulcerative colitis, or Crohn’s disease, the prevalence of B 27 drops to around 65%. A possible explanation is that the genes of inflammatory bowel conditions and psoriasis contribute to the development of spondylitis making the presence of B 27 less necessary. The varying clinical picture and the lower prevalence of
of B 27
B 27 when some of the associated diseases are present suggest that several genes are involved to help, hinder, or mask each other. On the clinical side both Csonka and A. Catin and colleagues reported that the long-term outlook was less favourable than indicated in some tests, with a 40% rate of late disability, predominantly due to chronic foot pain and less commonly to recurrent uveitis with loss of vision. T. Bitter and co-workers described a series of young adults with persistent yet reversible seronegative pauciarthritis in whom the prevalence of B 27 was 47.5%-i.e., somewhere between rheumatoid arthritis (RA) and Reiter’s syndrome. On American Rheumatism Association criteria this homogeneous group accounted for almost 90% of all "possible" or "probable" RA seen. Incomplete Reiter’s syndrome is not a happy description for this group, which seems to deserve a designation of its own. Csonka suggested that urethritis should always be excluded by a search for early-morning urethral pus, which may be missed after micturition. There was no consensus regarding the role of pathogens isolated initally from the genital or alimentary tract in Reiter’s syndrome. Although some years ago there was excitement after Chlamydia trachomatis had been found in the synovial fluid by Schachter,2 this seems to have been due to contamination in the laboratory. It is noteworthy that B 27 is a major antigen located on the short arm of human chromosome 6, a region which encodes immune responses. Environmental factors such as genital and alimentary bacteria may interact with immune reactions modulated by sex-dependent factors. Clearly our present limited knowledge indicates new directions for research. The finding of the B 27 association has raised many questions and we have reached the position where even a clear definition eludes us: as one speaker in Lausanne put it, "We all know what we are talking about but cannot define it".
MORALE AND COMMITMENT THE Government’s latest reactions in the discussions about N.H.S. consultant contracts (see p. 576) arise from a wish to improve "the morale of consultants in the N.H.S. and their commitment to it" (in Mr Jenkin’s words). The morale of some will surely be fortified by the proposed changes, but many whole-timers, especially those for whom private practice is less appealing or less available, may not feel all that uplifted. Their commitment to the N.H.S. has been and is total and these tinkerings with their contract can have little attraction for those who believe that the use of private practice as a morale booster is incompatible with the strengthening of commitment to the N.H.S. or with raising its standards. 2. Schachter
patients
J, Barnes MG, Jones JP. Isolation of Bedsoniæ from the joints of with Reiter’s syndrome. Proc Soc Exp Biol 1966; 122: 283-85.