Radiological and Familial Aspects

Size: px

Start display at page:

Download "Radiological and Familial Aspects"

Lambert Grant
6 years ago
Views:

46 Proc. roy. Soc. Med. Volume 67 January 1974 2 shows shrunken fibrotic upper lobes with a mycetoma in the right apical cavity (Figs 1 and 2).

1 46 Proc. roy. Soc. Med. Volume 67 January shows shrunken fibrotic upper lobes with a mycetoma in the right apical cavity (Figs 1 and 2). He has strongly positive precipitating antibodies to Aspergillusfumigatus. Skin tests to this fungus are negative. Spinal X-rays show advanced changes of ankylosing spondylitis. Respiratory function tests show total lung capacity of 3.1 litres (predicted 6.2), FEV1 1.3 litres (predicted 3.2), VC 1.3 litres (predicted 4.2), arterial Po2 85 mmhg, arterial Pco2 55 mmhg, arterial ph 7.30, transfer factor (single breath method) 14 ml per min per mmhg (predicted 27). Discussion The first description of the association of ankylosing spondylitis and nontuberculous pulmonary fibrosis was made by Hamilton (1949). A review of this association by Davies (1972), revealed that there were over 50 case reports and Davies, himself, added a further 7 patients. The pulmonary fibrosis commences in long-standing spondylitics with the onset of cough, sputum, and dyspncea. A diffuse mottling is seen in the upper zones of the lung fields which eventually progresses to dense fibrosis of the upper lobes, cavity formation, and bronchiectasis. Pleural thickening occurs over the involved areas. Haemoptyses are common, especially so with the development of aspergillomas in the lung cavities. These patients, not surprisingly, are often misdiagnosed as suffering from pulmonary tuberculosis, but, as in this patient, antituberculous drugs are ineffective. There is probably no special predisposition to pulmonary tuberculosis in ankylosing spondylitis (Wilkinson & Bywaters 1958, Zorab 1962, Hart et al. 1963) and there is no evidence that patients with -nontuberculous fibrosis are more liable to develop tuberculosis (Davies 1972). Histological examination of nontuberculous pulmonary fibrosis reveals non-specific histological features. The alveolar walls are thickened, with interstitial fibrosis; occasionally giant cells of foreign body type are seen, and large numbers of histiocytes may be found in the alveoli; there is no evidence of a vasculitis (M 0 Skelton 1973, personal communication). There is at present no effective treatment. Acknowledgments: I am grateful to Dr Paul Forgacs, Brook Hospital, for carrying out the respiratory function tests. REFFERENCES Davies D (1972) Quarterly Journal ofmedicine 41, 395 Hamilton K A (1949) Annals ofinternal Medicine 31, 216 Hart F D, Emerson P & Gregg I (1963) Annals ofofthe Rheumatic Diseases 22, 11 Wilkinson M & Bywaters E G L (1958) Annals ofthe Rheumatic Diseases 17, 209 Zorab P A (1962) Quarterly Journal ofmedicine 31, 267 Psoriatic Spondylitis: Clinical, Radiological and Familial Aspects by J M H Moll DM MRCP (Sheffield Centre for the Investigation and Treatment ofrheumatic Diseases, Nether Edge Hospital, Sheffield, Sl1 9EL) The association between psoriasis and peripheral arthritis has been recognized since the early nineteenth century (Alibert 1818). However, the significance of the association between psoriasis and ankylosing spondylitis was reported for the first time much more recently (Zellner 1928). A number of further reports followed in the 1930s (Bauer & Vogl 1931, Weissenbach 1938, Dawson & Tyson 1938, Epstein 1939), but it was only in the 1950s that the concept of 'psoriatic spondylitis' as a specific entity became more widely accepted (Vilanova & Pifiol 1951, Sherman 1952, Fletcher & Rose 1955, Graber-Duvernay 1957, Wright 1957, Coste et al. 1958). There is still no universally agreed definition of psoriatic spondylitis (spondylitis psoriatica, psoriasis spondylitica, spondylarthrite psoriasique, psoriasis-spondylarthritis), but at present it is regarded by some workers as an alternative expression of psoriatic arthritis (Baker et al. 1964, Moll 1971, Moll & Wright 1973b). Until properly evaluated criteria for the syndrome can be established, the author suggests the following as a provisional definition for psoriatic spondylitis: 'the association of skin and/or nail psoriasis and ankylosing spondylitis - definite ankylosing spondylitis according to New York criteria (Bennett & Wood with or without seronegative peripheral inflammatory arthritis'. Psoriatic spondylitis, thus defined, exhibits a wide spectrum of clinical presentations. Based on a personal study of 40 patients, four categories may be delineated: (I) Patients with psoriasis and ankylosing spondylitis withoutperipheral arthritis. (2) Patients with psoriasis, peripheral arthritis and ankylosing spondylitis in which the spondylitis predominates. (3) Patients with psoriasis, peripheral arthritis and ankylosing spondylitis in which the peripheral arthritis predominates. (In these patients, clinical features referable to the spine are often minimal or absent, and involvement of the sacroiliac joints or spine is usually found only on routine radiography.) (4) Patients with psoriasis, peripheral arthritis and ankylosing spondylitis in whom involvement of peripheral and axial joints is of roughly equal severity.

3 Section ofphysical Medicine 47 Fig I Arthritis mutilans in a patient with psoriatic spondylitis. Note the phalangeal osteolysis ('whittling).

Cervical spine showing ankylosis ofapophysealjoints, and osteoporosis Fig 4 Psoriatic spondylitis.

sacroiliitis in a patient with psoriatic spondylitis; B, Ankylosis ofthe sacroiliac joints in a patient with advancedpsoriatic spondylitis; note

and not simply the fortuitous association between psoriasis and idiopathic ankylosing spondylitis, stems largely from epidemiological work

2 3 Section ofphysical Medicine 47 Fig I Arthritis mutilans in a patient with psoriatic spondylitis. Note the phalangeal osteolysis ('whittling). Clinically, the patient had 'doigts-en-lorgnettces Fig 3 Psoriatic spondylitis. Cervical spine showing ankylosis ofapophysealjoints, and osteoporosis Fig 4 Psoriatic spondylitis. Note anterior andposterior bridging between L 3 and 4 (arrow), 'squaring' of lower lumbar vertebrae, and osteoporosis Fig 2 A, Bilateral sacroiliitis in a patient with psoriatic spondylitis; B, Ankylosis ofthe sacroiliac joints in a patient with advancedpsoriatic spondylitis; note the sacroiliac effacement and bilateral 'stars' in the upper third ofeach ghostjoint That psoriatic spondylitis is an entity in its own right, and not simply the fortuitous association between psoriasis and idiopathic ankylosing spondylitis, stems largely from epidemiological work demonstrating a significantly increased prevalence of ankylosing spondylitis or sacroiliitis in psoriatic patients (Graber-Duvernay 1957, Wright 1961, Reed 1961, Jajic 1968). The only reported study of the prevalence of psoriasis in ankylosing spondylitis failed to show a

3 48 Proc. roy. Soc. Med. Volume 67 January 1974 Table 1 Features distinguishing psoriatic spondylitis from idiopathic ankylosing spondylitis (various authors) Reference Bunim (1962) Bywaters & Dixon (1965) Jajic (1968) Langeland & Roaas (1971) Theiss et al. (1969) McEwen et al. (1971) Feature distinguishing psoriatic spondylitis from idiopathic ankylosing spondylitis Unusual paravertebral ossification (I) Large sacroiliac erosions (usurae) (2) Sclerosis more common in the ilium than in the sacrum (appearance similar to osteitis condensans ilii) (3) Asymmetrical sacroiliitis (4) Asymmetrical paravertebral ossification (5) Infrequency of 'bamboo' spine (6) Poor correlation between radiological appearances in spine and sacroiliac joints, and impairment ofspinal mobility (1) Solid fusion ofthoracic vertebrm, preceded by destruction ofadjacent vertebral bodies (2) Association between spondylitis and pustular psoriasis (1) Paraspinal ossification and atypical syndesmophytes (2) Frequent peripheral arthritis ofhands and feet (3) Tendency to hyperuricmmia (4) Late manifestation ofspondylitis in a quarter ofcases (5) Modest symptomatology (6) Favourable prognosis 'Other-than-marginal' syndesmophytes (non-marginal, 'tear-drop' or 'inverted comma', and Bywaters-Dixon forms) 4 statistically significant excess of psoriasis (5 of 267), but this was a retrospective study from hospital case records (Lefkovits & Thomas 1958). Further evidence to support the nosological individuality of psoriatic spondylitis, compared with idiopathic ankylosing spondylitis, stems from observations by Baker et al. (1963). These workers emphasized the following points to differentiate psoriatic from idiopathic spondylitis: onset in middle age, predominance among females, and gross involvement of small peripheral joints. A number of atypical sacroiliac and spinal features, recently reported in radiological studies ofpatients with psoriasis and spondylitis (Bunim 1962, Bywaters & Dixon 1965, Jajic 1968, Theiss et al. 1969, Langeland & Roaas 1971, McEwen et al. 1971), may be added to these points of differentiation. Clinical Features Qualitatively, the pattern of skin and nail psoriasis in patients with psoriatic spondylitis differs in no way from the rash in uncomplicated psoriasis and in psoriatic peripheral arthritis. However, in one series (Reed 1961) 60% of patients with psoriatic spondylitis had pustular psoriasis. In view of the close similarity between pustular psoriasis and keratoderma blenorrhagica, this observation provides additional evidence to support the concept of a relationship between psoriatic arthritis and Reiter's disease (Wright & Reed 1964, Wright & Moll 1971). The clinical features in the chest and spine (pani, stiffness, and limitation of movement) are the same in psoriatic spondylitis as in idiopathic ankylosing spondylitis, although Jajic (1968) found his patients to be relatively free of symptoms and spinal rigidity. Iritis and aortic disease have been reported in patients with psoriatic spondylitis as well as in uncomplicated spondylitis (Reed 1961). Four of Reed's 10 patients with psoriatic spondylitis had probable spondylitic heart disease, and in another study (Graham & Smythe 1958), 5 of 28 patients with spondylitic heart disease had psoriasis. The pattern of peripheral joint involvement, however, differs in the two diseases; in psoriatic Table 2 Prevalence of sacroiliitis in first-degree relatives of probands with psoriatic arthritis, in population controls, and in spouse controls Prevalence ofsacroiliitis (Grades 2-4) No. % Observed prevalence in 13/ relatives Expected prevalence: Population controls 3/ Spouse controls 1/ The degree offamilial aggregation calculated from population controls was 6.7 (P= 0.005) and that from spouse controls was 5.6 (P= 0.04) 0 1 F Macrae & J M H Moll (1970, unpublished) Table 3 Prevalence of sacroiliitis in first-degree relatives of probands with psoriasis and other arthritis, in population controls, and in spouse controls Prevalence ofsacroiliitis (Grades 2-4) No. % Observed prevalence 0/27 0 in relatives Expected prevalence: Population controls 3/ Spouse controls 1/ The familial aggregation was zero using both population and spouse controls

5 Section ofphysical Medicine 49 II I 11 ]IH Fig 5 Pedigree ofafamily in which the proband hadperipheralpsoriatic arthritis and the mother psoriatic spondylitis spondylitis, the tendency is towards

4 5 Section ofphysical Medicine 49 II I 11 ]IH Fig 5 Pedigree ofafamily in which the proband hadperipheralpsoriatic arthritis and the mother psoriatic spondylitis spondylitis, the tendency is towards involvement of small peripheral joints, sometimes with marked destruction (Fig 1). The patterns of peripheral joint involvement are similar to those seen in psoriatic arthritis without spondylitis (oligoarticular asymmetrical, 'rheumatoid', DIP, or mutilans patterns). By contrast, idiopathic ankylosing spondylitis is characterized by involvement of large and medium peripheral joints, particularly root joints (hips and shoulders). Another point of differentiation is that peripheral arthritis is much more common in psoriatic spondylitis: 71 % (Theiss et al. 1969), 82% (Graber-Duvernay 1957), 90% (Reed 1961). Radiological Features In most cases of psoriatic spondylitis, the radiological features of the sacroiliac joints and spine are similar to those found in idiopathic ankylosing spondylitis (Fletcher & Rose 1955, Graber- Duvernay 1957, Wright 1957, Avila et al. 1960, Dixon & Lience 1961, Kaplan et al. 1963, Baker et al. 1963, Estrin 1963). These changes are also similar to those seen in other forms of spondylitis, including the spondylitis associated with Reiter's disease, ulcerative colitis, Crohn's disease and Whipple's disease. Some typical radiological features of psoriatic spondylitis are shown in Figs 2-4. Some workers have described atypical sacroiliac and spinal features in psoriatic patients. These are summarized in Table 1. At present, it is difficult to assess the significance of these unusual spinal features in psoriatic subjects and further more extensive and controlled studies are needed to evaluate their specificity. Familial Features Graber-Duvemay (1957), and more recently Theiss et al. (1969), have drawn attention to familial aggregation in psoriatic spondylitis. The author, in a controlled family study of 108 Fig 6 Pedigree ofafamily in which the proband hadperipheralpsoriatic arthritis and the fatherpsoriatic spondylitis with peripheral arthritis Q- = Proband O o Confirmed = SACRO-ILIITIS = Reported \<v -sacro-ihiitis C - Confirmed PSORIASIS Reported psoriasis Key to Figs 5-9 d = Deceased Peripheral = PSORIATIC ARTHRITIS =.. plus * sacro-i itis Seroneg. cj = POLVARTHRITIS = Reported polyarthritis patients with psoriasis and arthritis (88 with true psoriatic arthritis and 20 with psoriasis and other arthritis) has found further evidence to support this phenomenon (Moll 1971, Moll & Wright 1973a). Prevalences of sacroiliitis and ankylosing spondylitis in patients with psoriatic arthritis and in patients with psoriasis and other arthritis are shown in Tables 2 and 3. The principal conclusion to be drawn from these data is that sacroiliitis (grades 2-4) is clustered (statistically significant compared with population and spouse controls) in first-degree relatives of probands with true psoriatic arthritis, but not in the relatives of probands with psoriasis and other arthritis (RA, OA, and gout). The fact that a significant difference was observed between relatives and spouse controls, as well as between relatives and population controls, suggests that genetic factors are involved in the familial aggregation of sacroiliitis in psoriatic arthritis families. Probands with psoriatic arthritis of spondylitic pattern did not have significantly more relatives with sacroiliitis or ankylosing spondylitis than probands without spondylitis. In some relatives with sacroiliitis or spondylitis, this was associated with psoriasis

5 50 Proc. roy. Soc. Med. Volume 67 January IR Fig 7 Pedigree ofafamily in which the proband had peripheralpsoriatic arthritis and the younger brother ankylosing spondylitis without psoriasis I Fig 8 Pedigree ofafamily in which theproband hadperipheralpsoriatic arthritis and spondylitis and thefather grade 2 bilateral sacroiliitis without clinicalfeatures II Fig 9 Pedigree ofafamilypreviously reported (Moll et al. 1973) in which the proband hadperipheral psoriatic arthritis, the identical triplet brother psoriatic spondylitis without peripheral arthritis, and the mother bilateral grade 3 sacroiliitis without clinicalfeatures. The non-identical triplet brother had neitherpsoriasis, arthritis nor spondylitis (Figs 5 and 6), in others it was not (Figs 7 and 8). Fig 9 shows an interesting pedigree which has been reported previoulsly (Moll et al. 1973) in which the identical triplet brother of a proband with psoriatic arthritis had psoriatic spondylitis, and the mother had sacroiliitis without psoriasis. Summary (1) The relationship between psoriasis and ankylosing spondylitis and/or sacroiliitis ('psoriatic spondylitis') probably reflects a specific clinical entity and not simply the chance association between psoriasis and idiopathic ankylosing spondylitis. (2) Psoriatic spondylitis is probably an alternative expression of psoriatic arthritis. (3) In most cases, the spondylitis of psoriatic subjects differs in no way, either clinically or radiologically, from that of idiopathic ankylosing spondylitis. (4) Familial evidence is presented which suggests that genetic factors may be involved in the association between psoriasis and ankylosing spondylitis. REFERENCES Alibert J L (1818) Precis Thdorique et Pratique sur les Maladies de la Peau. Caille et Ravier, Paris; 2, 21 Avila R, Pugh D G, Slocumb C H & Winkelmann R K (1960) Radiology 75, 691 Baker H, Golding D N & Thompson M (1963) Annals ofinternal Medicine 58, 909 (1964) British Journal ofdermatology 76, 549 Bauer J & Vogl A (1931) Klinische Wochenschrift 10, 1700 Bennett P H & Wood P H N (1968) Population Studies of the Rheumatic Diseases. Proceedings of the 3rd International Symposium, New York, Excerpta Medica Foundation, Amsterdam Bunim J J (1962) Annals ofinternal Medicine 57, 1018 Bywaters E G L & Dixon A St J (1965) Annals ofthe Rheumatic Diseases 24, 313 Coste F, Frangon J, Touraine R & Loyau G (1958) Revue du Rhumatisme et des Maladies Ostdo-Articulaires 25, 75 Dawson M H & Tyson T L (1938) Transactions ofthe Association of American Physicians 53, 303 Dixon A St J & Lience E (1961) Annals ofthe Rheumatic Diseases 20, 247 Epstein E (1939) Archives of Dernmatology and Syphilology 40, 547 Estrin I J (1963) Arthritis and Rheumatism 6, 268 Fletcher E & Rose F C (1955) Lancet i, 695 Graber-Duvernay J (1957) Revue du Rhumatisme et des Maladies Ostdo-Articulaires 24, 288 Graham D C & Smythe H A (1958) Bulletin on Rheumatic Diseases 9, 171 Jajid 1 (1968) Annals ofthe Rheumatic Diseases 27, 1 Kaplan D A, Platz C M, Nathanson L & Frank L (1963) Arthritis and Rheumatism 6, 281 Langeland N & Roaas A (1971) Acta orthopaedica Scandinavica 42, 391 Lefkovits A M & Thomas J R (1958) Annals ofinternal Medicine 49, 89 McEwen C, DiTata D, Lingg C, Porini A, Good A & Rankin T (1971) Arthritis and Rheumatism 14, 291 Moll J M H (1971) DM Thesis, Oxford Moll J M H, Johnson G & Wright V (1973) Annals ofrheumatology and Rehabilitation (in press) Moll J M H & Wright V (1973a) Annals ofthe Rheumatic Diseases 32, 181 (1973b) Seminars in Arthritis and Rheumatism 3, 35 Reed W B (1961) Acta Dermato- Venereologica 41, 396 Sherman M (1952) Journal ofbone andjoint Surgery 34A, 831 Theiss B, Boni A, WagenhaUser F, Schnyder U W & Fehr K (1969) Zeitschriftftr Rheumaforschung 28, 93 Vilanova X & Piflol J (1951) Rheumatism 7, 197 Weissenbach R-J (1938) Archives dermato-syphiligraphiques de la Clinique de l'h6pital Saint-Louis 10, 13 Wright V (1957) British Journal ofradiology 30, 113 (1961) Annals ofthe Rheumatic Diseases 20, 123 Wright V & Moll J M H (1971) Bulletin on Rheumatic Diseases 21, 627 Wright V & Reed W B (1964) Annals ofthe Rheumatic Diseases 23, 12 Zellner E (1928) Mfinchener medizinische Wochenschrift 75, 903

Psoriatic Spondylitis: A Clinical and Radiological Description of the Spine in Psoriatic Arthritis

Quarterly Journal of Medicine, New Series XLVI, No. 8, pp. -5, October 977 Psoriatic Spondylitis: A Clinical and Radiological Description of the Spine in Psoriatic Arthritis J. R. LAMBERT AND V. WRIGHT