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Home , Spondyloarthropathy

LETTERS TO THE EDITOR 155

indicates increased risk for microvascular disease. N murmur was audible along the left sternal border EnglJMed 1981;305:191-4. and/or a typical diastolic Doppler signal was 4. Pal B, Anderson J, Griffiths ID, Dick WC. Rheumatic demonstrated under the aortic valve on the Echo- disorders in diabetes mellitus: association between lim- ited joint mobility and Dupuytren's Doppler as well as a diastolic fluttering of the [Abstract]. Br J Rheumawl 1986;25:122-3. anterior leaflet of the mitral valve. Severity of AI 5. Prokop DJ, Tuderman L, Guzman NA. Collagen in was arbitrarily assessed in relation to clinical and normal and diseased connective tissue. In: McCarty echocardiographic data. AI was considered as mild JD, ed. and allied conditions. 9th edn. Phila- in asymptomatic patients if auscultation and Echo- delphia: Lea & Febiger, 1979. Doppler revealed a typical AI in the absence of an 6. Buckingham BA, Vitto J, Sandbork C, et al. increased left ventricular cavity. The Doppler sig- Scleroderma like syndrome and non-enzymatic glyco- nal of regurgitation had to be recorded in the left sylation of collage in children with poorly controlled ventricle only below the aortic leaflets. It was con- insulin dependent diabetes [Abstract]. Paediatr Res 1981;5:626. sidered severe when patients were symptomatic, 7. Schnider SL, Kohn RR. Effects of age and diabetes on the left ventricular cavity enlarged and AI recorded the solubility and non-enzymatic glycosylation of in the left ventricle beyond the tip of the mitral human skin collage. J Clin Invest 981;67:1630-5. valve. Conventional echocardiographic criteria for 8. Bollinger A,FreyJ, JagerK,era/. Patterns of diffusion mitral valve prolapse (MVP) were applied [9]. In a through skin capillaries in patients with longterm strictly perpendicular M-mode echocardiographic diabetes. N EnglJ Med 1982;307:1305-20. examination, a minimum 3 mm posterior bulging to the left atrium of the echoes of one or both mitral Clinical and Echocardiographic Assessment in leaflets was requested. In the apical-view examin- Juvenile-onset ation, a minimum 3 mm posterior bulging to the left atrium of one or both leaflets was requested .Atten- SIR—Aortic incompetence (AI) has been noted to tion was also paid to accessory signs such as thick- develop in adults with ankylosing (AS), ness and protomesosystolic anterior motion of the Reiter's syndrome (RS) and other subsets of spon- mitral valve. dyloarthritis [1]. It is considered uncommon in juvenile cases and only 10 cases have been reported None of the patients had signs of cardiac failure. [2-8]. M-mode and two-dimensional echocardio- A faint early diastolic murmur along the upper left graphy with pulsed Doppler (Echo-Doppler) were sternal border was reported in three cases 2 used to determine whether changes of the aortic months, and 4, 5 and 7 years after arthritis was root or valve could be identified in all the juvenile- diagnosed. Aortic regurgitation was confirmed by onset spondyloarthritis patients seen by our rheu- Echo-Doppler in all three cases. AI was severe in matology unit over a 7-year period. There were 20 one and mild in two. In the 17 cases with normal cases (Table III) referred from within our own auscultation, Echo-Doppler revealed in a 10-year- small region. Their present ages range from 7 to 36 old girl, with a 7-year history of rheumatic com- (average 17) years, the age at onset of their rheu- plaints, minimal AI in a tricuspid aortic valve. matic disease was between 3 and 16 (average 12) Clinically silent MVP was detected in eight patients years and the mean duration of follow-up was 5 (Table III), two of them having AI. None of the years. Five had obvious clinical and radiological cases with MVP had reduced chest expansion, ver- signs of AS, five had complete or incomplete RS tebral syndesmophytes, pectus excavatum, severe and none had psoriatic or intestinal . In , straight back hypermobility or Marfan's 10 there was a pauciarticular onset without back syndrome. None had pericardial effusion or thick- pain, or extra-articular manifestations. ening. Left ventricular dimensions and perform- HLA-B27 phenotype was found in 19. ances were normal in all but one case with AI. No AI was diagnosed when a typical early diastolic atrioventricular conduction defects were observed on ECG. Anterior occurred in only one patient who had no cardiac abnormality. All cases TABLE III with AI and/or MVP possessed HLA-B27 antigen. INCIDENCE OF AORTIC INCOMPETENCE AND MITRAL VALVE PROLAPSE IN JUVENILE ONSET SPONDYLOARTHROPATHY The occurrence of AI in juvenile-onset AS has been known for a considerable time [3] and in Aortic Mitral adults, the incidence varies between 0.9 and 10%. Diagnosis No. of Males incom- valve In conventional ECG recordings, none of our cases cases (B27 petence prolapse had the atrioventricular conduction defects which +ve) No (%) No (%) occur in adult onset AS. AI developed in one case Ankylosing within as little as 2 months of arthritis and in the spondylitis 5 (4) 5 1(20) 1(20) other two after several years. Murmurs of AI have Reiter's been reported to occur after 10 months [2], 1 year syndrome 5 (5) 4 — 2(40) [3, 8] and 4 years [5] of arthritis. AI has also been Pauciarticular shown to antedate the onset of arthritis in an ado- arthritis 10 (10) 7 3(33) 5(50) lescent [4]. Total 20 (19) 16 4(20) 8(40) Our data are consistent with AI being a frequent 156 BRITISH JOURNAL OF VOL. XXVI NO. 2 finding in juvenile-onset spondyloarthropathy. It conjunctivitis, mucocutaneous lesions and arthri- appears to be well tolerated and left ventricular tis. It generally runs a protracted clinical course hypertrophy was found in only one patient. How- with exacerbations and remissions but less fre- ever, rapid progression with aortic valve replace- quently patients develop a chronic severely ment has been documented [4]. debilitating illness. There is considerable clinical The recording of eight cases (40%) of MVP was overlap between RS and unexpected. However, our diagnostic criteria of (AS) and both are strongly associated with the MVP may be oversensitive. If we had applied later HLA-B27 antigen [1,2]. However, whereas up to criteria [10], MVP would have been diagnosed in 10% of AS individuals may develop amyloidosis none of our patients. [3], it is rarely seen in RS and only three cases have Six of the patients with MVP were male, which is been reported [4-6]. This is surprising in view of the surprising since there is a known female predomi- similarities between the two conditions and the pro- nance. Two had a combination with AI. None of longed severe often observed in RS. the acknowledged clinical associations of MVP We report here the case of a man with a severe, were encountered, raising the possibility that it chronic, multisystem disease who developed could be causally related to spondyloarthropathy. amyloidosis and the nephrotic syndrome 14 years The recognition of AI and MVP is important after the onset of RS. since these valves may be predisposed to infective A 32-year-old Australian white male was admit- endocarditis and AI may become severe enough to ted for investigation of nephrotic syndrome. He require valve replacement. was well until 14 years earlier when, after an epi- J. C. GERSTER1 sode of urethritis, thought to be gonorrhoea, he M. PAYOT2 was treated with penicillin. Four weeks later he P. PICCININ1 developed arthritis in the proximal interphalangeal •Rheumatology and Rehabilitation Centre, and joint of an index finger, both ankles, right knee, left 2Paediatric Cardiology Department, University elbow and bilateral . A diagnosis of Hospital, Chuv, 1011-Lausanne, Switzerland. RS was made and he was commenced on phenyl- butazone 100 mg b.d. Two years later he suffered a 1. Ball J. Pathology and pathogenesis. In: Moll JMH, severe episode of anterior uveitis which failed to ed. Ankylosing spondylitis. Edinburgh: Churchili respond to topical and systemic steroid therapy and Livingstone, 1980;96-112. 2. Kean WF, Anastassiades TP, Ford PM. Aortic incom- one eye was eventually enucleated. petence in HLA B27 positive juvenile arthritis. Ann Over the next two years, the patient had per- Rheum Dis 1980;39:294-5. sistent disease activity, which was poorly respon- 3. Bulkley BH, Roberts WC. Ankylosing spondylitis sive to nonsteroidal anti-inflammatory agents and and aortic regurgitation: description of the charac- required systemic corticosteroid therapy (beta- teristic cardiovascular lesion from study of eight nec- methasone 0.5 mg b.d.). This failed to give ropsy patients. Circulation 1973;48:1014-27. adequate disease control and he developed promi- 4. Stewart SR, Robbins DL, Castles JJ. Acute fulminant aortic and mitral insufficiency in ankylosing spon- nent signs of Cushing's syndrome including hyper- dylitis. N EnglJ Med 1978;229:1448-9. tension (BP 160/110). Azathioprine was 5. Reid GD, Patterson MWH, Patterson AC, el al. commenced (200 mg mane) and corticosteroids Aortic insufficiency in association with juvenile were reduced (prednisolone 5 mg/day). Synovec- ankylosing spondylitis. / Pediatr 1979;95:78-80. tomies of both knees were performed followed by 6. Ansell BM. Juvenile spondylitis and related dis- bilateral hip replacement and arthroplasty of one orders. In: Moll JMH, ed. Ankylosing spondylitis. knee 10 and 13 years after disease onset. The Edinburgh: Churchill Livingstone, 1980;12O-36. patient was severely incapacitated by his chronic 7. Rosenberg AM, Petty RE. A syndrome of seronega- . tive and arthropathy in children. Arthritis Rheum 1982;25:1041-7. There was a family history of arthritis which 8. Pelkonen P, Byring R, Pesonen E, el al. Rapidly affected the patient's father and older brother, but progressive aortic incompetence in juvenile ankylos- no history of iritis or amyloidosis. On admission he ing spondylitis: a case report. Arthritis Rheum 1984; was wasted with cushingoid facies and there was 27:698-700. prominent synovitis involving shoulders, elbows, 9. MarkiewiczW, Stoner J, London E,etal. Mitralvalve wrists, all metacarpophalangeal joints, knees, prolapse in one hundred presumably healthy young ankles and feet. Marked spinal and loss of females. Circulation 1976,53:464-73. 10. Warth DC, King ME, Cohen JM, Tesoriero VL, Mar- lumbar were evident. There was a hyper- cus E, Weyman AE. Prevalence of mitral valve pro- keratotic, scaly rash over the ankles and calves but lapse in normal children. J Am Coll Cardiol 1985;5: no evidence of psoriasis. Abdominal examination 1173-7. revealed hepatomegaly and 3 cm of non-tender splenomegaly but the kidneys were not palpable. He was mildly hypertensive with ankle oedema but Reiter's Syndrome and Amyloidosis no other features of heart failure were present. SIR—Reiter's syndrome (RS) is a well-charac- Fundoscopy revealed grade II hypertensive reti- terized symptom complex consisting of urethritis, nopathy. His Hb was 12.2 g/dl, WBC 8.1x10'/!,