GERSTER, J. C.

PAYOT, M.

PICCININ, P.

RERO DOC Digital Library

LETTERS TO THE EDITOR 155indicates increased risk for microvascular disease. NEnglJMed 1981;305:191-4.4. Pal B, Anderson J, Griffiths ID, Dick WC. Rheumaticdisorders in diabetes mellitus: association between lim-ited joint mobility and Dupuytren's contracture[Abstract]. Br J Rheumawl 1986;25:122-3.5. Prokop DJ, Tuderman L, Guzman NA. Collagen innormal and diseased connective tissue. In: McCartyJD, ed. Arthritis and allied conditions. 9th edn. Phila-delphia: Lea & Febiger, 1979.6. Buckingham BA, Vitto J, Sandbork C, et al.Scleroderma like syndrome and non-enzymatic glyco-sylation of collage in children with poorly controlledinsulin dependent diabetes [Abstract]. Paediatr Res1981;5:626.7. Schnider SL, Kohn RR. Effects of age and diabetes onthe solubility and non-enzymatic glycosylation ofhuman skin collage. J Clin Invest 981;67:1630-5.8. Bollinger A,FreyJ, JagerK,era/. Patterns of diffusionthrough skin capillaries in patients with longtermdiabetes. N EnglJ Med 1982;307:1305-20.Clinical and Echocardiographic Assessment inJuvenile-onset SpondyloarthropathySIR—Aortic incompetence (AI) has been noted todevelop in adults with ankylosing spondylitis (AS),Reiter's syndrome (RS) and other subsets of spon-dyloarthritis [1]. It is considered uncommon injuvenile cases and only 10 cases have been reported[2-8]. M-mode and two-dimensional echocardio-graphy with pulsed Doppler (Echo-Doppler) wereused to determine whether changes of the aorticroot or valve could be identified in all the juvenile-onset spondyloarthritis patients seen by our rheu-matology unit over a 7-year period. There were 20cases (Table III) referred from within our ownsmall region. Their present ages range from 7 to 36(average 17) years, the age at onset of their rheu-matic disease was between 3 and 16 (average 12)years and the mean duration of follow-up was 5years. Five had obvious clinical and radiologicalsigns of AS, five had complete or incomplete RSand none had psoriatic or intestinal arthropathy. In10 there was a pauciarticular onset without backpain, sacroiliitis or extra-articular manifestations.HLA-B27 phenotype was found in 19.AI was diagnosed when a typical early diastolicTABLE IIIINCIDENCE OF AORTIC INCOMPETENCE AND MITRAL VALVEPROLAPSE IN JUVENILE ONSET SPONDYLOARTHROPATHYDiagnosisAnkylosingspondylitisReiter'ssyndromePauciarticulararthritisTotalNo. ofcases (B27+ve)5 (4)5 (5)10 (10)20 (19)Males54716Aorticincom-petenceNo (%)1(20)—3(33)4(20)MitralvalveprolapseNo (%)1(20)2(40)5(50)8(40)murmur was audible along the left sternal borderand/or a typical diastolic Doppler signal wasdemonstrated under the aortic valve on the Echo-Doppler as well as a diastolic fluttering of theanterior leaflet of the mitral valve. Severity of AIwas arbitrarily assessed in relation to clinical andechocardiographic data. AI was considered as mildin asymptomatic patients if auscultation and Echo-Doppler revealed a typical AI in the absence of anincreased left ventricular cavity. The Doppler sig-nal of regurgitation had to be recorded in the leftventricle only below the aortic leaflets. It was con-sidered severe when patients were symptomatic,the left ventricular cavity enlarged and AI recordedin the left ventricle beyond the tip of the mitralvalve. Conventional echocardiographic criteria formitral valve prolapse (MVP) were applied [9]. In astrictly perpendicular M-mode echocardiographicexamination, a minimum 3 mm posterior bulging tothe left atrium of the echoes of one or both mitralleaflets was requested. In the apical-view examin-ation, a minimum 3 mm posterior bulging to the leftatrium of one or both leaflets was requested .Atten-tion was also paid to accessory signs such as thick-ness and protomesosystolic anterior motion of themitral valve.None of the patients had signs of cardiac failure.A faint early diastolic murmur along the upper leftsternal border was reported in three cases 2months, and 4, 5 and 7 years after arthritis wasdiagnosed. Aortic regurgitation was confirmed byEcho-Doppler in all three cases. AI was severe inone and mild in two. In the 17 cases with normalauscultation, Echo-Doppler revealed in a 10-year-old girl, with a 7-year history of rheumatic com-plaints, minimal AI in a tricuspid aortic valve.Clinically silent MVP was detected in eight patients(Table III), two of them having AI. None of thecases with MVP had reduced chest expansion, ver-tebral syndesmophytes, pectus excavatum, severescoliosis, straight back hypermobility or Marfan'ssyndrome. None had pericardial effusion or thick-ening. Left ventricular dimensions and perform-ances were normal in all but one case with AI. Noatrioventricular conduction defects were observedon ECG. Anterior uveitis occurred in only onepatient who had no cardiac abnormality. All caseswith AI and/or MVP possessed HLA-B27 antigen.The occurrence of AI in juvenile-onset AS hasbeen known for a considerable time [3] and inadults, the incidence varies between 0.9 and 10%.In conventional ECG recordings, none of our caseshad the atrioventricular conduction defects whichoccur in adult onset AS. AI developed in one casewithin as little as 2 months of arthritis and in theother two after several years. Murmurs of AI havebeen reported to occur after 10 months [2], 1 year[3, 8] and 4 years [5] of arthritis. AI has also beenshown to antedate the onset of arthritis in an ado-lescent [4].Our data are consistent with AI being a frequent156 BRITISH JOURNAL OF RHEUMATOLOGY VOL. XXVI NO. 2finding in juvenile-onset spondyloarthropathy. Itappears to be well tolerated and left ventricularhypertrophy was found in only one patient. How-ever, rapid progression with aortic valve replace-ment has been documented [4].The recording of eight cases (40%) of MVP wasunexpected. However, our diagnostic criteria ofMVP may be oversensitive. If we had applied latercriteria [10], MVP would have been diagnosed innone of our patients.Six of the patients with MVP were male, which issurprising since there is a known female predomi-nance. Two had a combination with AI. None ofthe acknowledged clinical associations of MVPwere encountered, raising the possibility that itcould be causally related to spondyloarthropathy.The recognition of AI and MVP is importantsince these valves may be predisposed to infectiveendocarditis and AI may become severe enough torequire valve replacement.J. C. GERSTER1M. PAYOT2P. PICCININ1•Rheumatology and Rehabilitation Centre, and2Paediatric Cardiology Department, UniversityHospital, Chuv, 1011-Lausanne, Switzerland.1. Ball J. Pathology and pathogenesis. In: Moll JMH,ed. Ankylosing spondylitis. Edinburgh: ChurchiliLivingstone, 1980;96-112.2. Kean WF, Anastassiades TP, Ford PM. Aortic incom-petence in HLA B27 positive juvenile arthritis. AnnRheum Dis 1980;39:294-5.3. Bulkley BH, Roberts WC. Ankylosing spondylitisand aortic regurgitation: description of the charac-teristic cardiovascular lesion from study of eight nec-ropsy patients. Circulation 1973;48:1014-27.4. Stewart SR, Robbins DL, Castles JJ. Acute fulminantaortic and mitral insufficiency in ankylosing spon-dylitis. N EnglJ Med 1978;229:1448-9.5. Reid GD, Patterson MWH, Patterson AC, el al.Aortic insufficiency in association with juvenileankylosing spondylitis. / Pediatr 1979;95:78-80.6. Ansell BM. Juvenile spondylitis and related dis-orders. In: Moll JMH, ed. Ankylosing spondylitis.Edinburgh: Churchill Livingstone, 1980;12O-36.7. Rosenberg AM, Petty RE. A syndrome of seronega-tive enthesopathy and arthropathy in children.Arthritis Rheum 1982;25:1041-7.8. Pelkonen P, Byring R, Pesonen E, el al. Rapidlyprogressive aortic incompetence in juvenile ankylos-ing spondylitis: a case report. Arthritis Rheum 1984;27:698-700.9. MarkiewiczW, Stoner J, London E,etal. Mitralvalveprolapse in one hundred presumably healthy youngfemales. Circulation 1976,53:464-73.10. Warth DC, King ME, Cohen JM, Tesoriero VL, Mar-cus E, Weyman AE. Prevalence of mitral valve pro-lapse in normal children. J Am Coll Cardiol 1985;5:1173-7.Reiter's Syndrome and AmyloidosisSIR—Reiter's syndrome (RS) is a well-charac-terized symptom complex consisting of urethritis,conjunctivitis, mucocutaneous lesions and arthri-tis. It generally runs a protracted clinical coursewith exacerbations and remissions but less fre-quently patients develop a chronic severelydebilitating illness. There is considerable clinicaloverlap between RS and ankylosing spondylitis(AS) and both are strongly associated with theHLA-B27 antigen [1,2]. However, whereas up to10% of AS individuals may develop amyloidosis[3], it is rarely seen in RS and only three cases havebeen reported [4-6]. This is surprising in view of thesimilarities between the two conditions and the pro-longed severe inflammation often observed in RS.We report here the case of a man with a severe,chronic, multisystem disease who developedamyloidosis and the nephrotic syndrome 14 yearsafter the onset of RS.A 32-year-old Australian white male was admit-ted for investigation of nephrotic syndrome. Hewas well until 14 years earlier when, after an epi-sode of urethritis, thought to be gonorrhoea, hewas treated with penicillin. Four weeks later hedeveloped arthritis in the proximal interphalangealjoint of an index finger, both ankles, right knee, leftelbow and bilateral plantar fasciitis. A diagnosis ofRS was made and he was commenced on phenyl-butazone 100 mg b.d. Two years later he suffered asevere episode of anterior uveitis which failed torespond to topical and systemic steroid therapy andone eye was eventually enucleated.Over the next two years, the patient had per-sistent disease activity, which was poorly respon-sive to nonsteroidal anti-inflammatory agents andrequired systemic corticosteroid therapy (beta-methasone 0.5 mg b.d.). This failed to giveadequate disease control and he developed promi-nent signs of Cushing's syndrome including hyper-tension (BP 160/110). Azathioprine wascommenced (200 mg mane) and corticosteroidswere reduced (prednisolone 5 mg/day). Synovec-tomies of both knees were performed followed bybilateral hip replacement and arthroplasty of oneknee 10 and 13 years after disease onset. Thepatient was severely incapacitated by his chronicpolyarthritis.There was a family history of arthritis whichaffected the patient's father and older brother, butno history of iritis or amyloidosis. On admission hewas wasted with cushingoid facies and there wasprominent synovitis involving shoulders, elbows,wrists, all metacarpophalangeal joints, knees,ankles and feet. Marked spinal kyphosis and loss oflumbar lordosis were evident. There was a hyper-keratotic, scaly rash over the ankles and calves butno evidence of psoriasis. Abdominal examinationrevealed hepatomegaly and 3 cm of non-tendersplenomegaly but the kidneys were not palpable.He was mildly hypertensive with ankle oedema butno other features of heart failure were present.Fundoscopy revealed grade II hypertensive reti-nopathy. His Hb was 12.2 g/dl, WBC 8.1x10'/!,

Clinical and Echocardiographic Assessment in Juvenile-onset Spondyloarthropathy

Abstract

Similar works

Full text

Available Versions

RERO DOC Digital Library