210 Bentley, McAuliffe

14 Habenicht A J R, Goerig M, Grulich J, et al. Human platelet-derived growth 23 Gordon D, Bray M A, Morley J. Control of lymphokine secretion by factor stimulates prostaglandin synthesis by activation and by rapid de novo prostaglandins. Nature 1976; 262: 401-2. synthesis of cyclooxygenase. J Clin Invest 1985; 75: 1381-7. 24 Knudsen P J, Dinarello C A, Strom T B. Prostaglandins posttranscriptionally 15 Heldin C H, Backstrom G, Ostman A, et al. Binding of different dimeric inhibit monocyte expression of interleukin 1 activity by increasing intra- forms of PDGF to human fibroblasts: evidence for two separate receptor cellular cyclic adenosine monophosphate. J Immunol 1986; 137: 3189-94. types. EMBO7 1988; 7: 1387-94. 25 Kunkel S L, Spengler M, May M A, Spengler R, Larrick J, Rennick D. Ann Rheum Dis: first published as 10.1136/ard.49.4.210 on 1 April 1990. Downloaded from 16 Nister M, Hammacher A, Melistrom K, et al. A glioma-derived PDGF A Prostaglandin E2 regulates macrophage-derived tumor necrosis factor gene chain homodimer has different functional activities than a PDGF AB expression. Biol Chem 1988; 263: 5380-4. heterodimer purified from human platelets. Cell 1988; 52: 791-9. 26 Rola-Pleszczynski M, Lemaire I. Leukotrienes augment interleukin 1 17 Brom J, Knoller J, Koller M, Konig W. Tumour necrosis factors modulate production by human monocytes. J Immunol 1985; 135: 3958-61. the affinity state of the leukotriene B4 receptor on human neutrophils. 27 Rola-Pleszczynski M. Immunoregulation by leukotrienes and other lipoxy- Immunology 1988; 65: 647-9. genase metabolites. Immunology Today 1985; 6: 302-6. 18 Dewhirst F E, Ago J M, Peros W J, Stashenko P. Synergism between 28 Shirakawa F, Yamashita U, Chedid M, Mizel S B. Cyclic AMP-an parathyroid hormone and interleukin 1 in stimulating bone resorption in intracellular second messenger for interleukin 1. Proc Natl Acad Sci USA organ culture. 7ournal of Bone Mineral Research 1987; 2: 127-34. 1988; 85: 8201-5. 19 Akahoshi T, Oppenheim J J, Matsushima K. Interleukin- 1 stimulates its own 29 Zhang Y, Lin J X, Yip Y K, Vilcek J. Enhancement of cAMP levels and of receptor expression on human fibroblasts through the endogenous production protein kinase activity by tumor necrosis factor and interleukin 1 in human of prostaglandins. J Glin Invest 1988; 82: 1219-24. fibroblasts: role in the induction of interleukin 6. Proc Natl Acad S'ci USA 20 Elias J A. Tumor necrosis factor interacts with interleukin- 1 and interferons 1988; 85: 6802-5. to inhibit fibroblast proliferation via fibroblast prostaglandin-dependent and 30 Dayer J M, Goldring S R, Robinson D R, Krane S M. Effects of human prostaglandin-independent mechanisms. Am Rev Respir Dis 1988; 138: mononuclear cell factor on cultured rheumatoid synovial cells. Interactions 652-8. of prostaglandin E2 and cyclic adenosine 3'5-monophosphate. Biochem 21 Browning J. Interferons and rheumatoid arthritis: Insight into interferon Biophys Acta 1979; 586: 87-105. biology? Immunology Today 1987; 8: 3724. 31 Johnson H M, Russell J K, Torres B A. Second messenger role of arachidonic 22 Hart P H, Whitty G A, Piccoli D S, Hamilton J A. Control by IFN-y and acid and its metabolites in interferon-y production. J Immunol 1986; 137: PGE2 of TNF( and IL-l production by human monocytes. Immunology 3053-6. 1989; 66: 376-83. 32 Bourne H R. Who carries what message? Nature 1989; 337: 504-5.

Pigmented villonodular synovitis

In 1941 Jaffe, Lichtenstein, and Sutro introduced the term There have been many attempts to incriminate trauma as pigmented villonodular synovitis (PVNS) to describe a the cause, but no study clearly validates this claim. Many 'yellow-brown tumour-like tenosynovial lesion'.' Before varied substances have been injected into the joints of their paper this condition had gone under a variety of experimental animals, but none has ever reproduced the names, many of which implied a neoplastic disorder. Indeed typical clinical or histological features of PVNS. Myers et al the earliest descriptions assumed it was a malignant disease, felt that chronic repetitive trauma and haemarthrosis were and amputation was commonly practised. The first report importantfactors. The condition is not seen in haemophiliacs, was by Chassaignac in 1852, who described a nodular lesion however, nor in patients with Charcot's joints. No organisms occurring in the flexor tendons of the hand.2 In 1909 Moser have ever been consistently isolated from a lesion of PVNS, reported a diffuse type of lesion in the ankle.3 It was not though there is one isolated report of the finding of a until the paper of Jaffe et al, however, that a clear overall myxovirus-like structure from the knee in an unusual http://ard.bmj.com/ description of the condition was given. bilateral case.5 The likelihood of an infective cause seems Reporting on 20 of their own cases affecting joints, remote. tendon sheaths, and bursae, Jaffe et al defined them as The most likely cause seems to be inflammatory, but the either circumscribed or diffuse and showed that the noxious agent remains unidentified. Lipids have been histological appearances were similar in both types of suggested as they are seen in increased concentration articular synovitis and also in pigmented villonodular intracellularly in PVNS. There is no evidence for any tenosynovitis and bursitis. They described the salient systemic lipid abnormality, but Hirohata suggested a on October 1, 2021 by guest. Protected copyright. histological features: deposition of haemosiderin and infil- localised metabolic disturbance as the source of the lipids.6 tration of histiocytes and giant cells in a fibrous stroma Intra-articular injection of lipids does not induce PVNS, within the synovium of tendon sheaths and large joints. however, and probably, as Jaffe pointed out, the presence of They suggested that the condition represented an inflam- lipids in the lesions is a secondary phenomenon as seen in matory response to an unknown agent. In their experience other inflammatory lesions around bone. the treatment of choice was complete excision, and recur- Most authors since Jaffe have thought that PVNS is a rence was due to inadequate removal of diseased tissue. non-neoplastic process, but there are still some dissenters.7 Recurrences were never malignant and could be adequately Certainly the condition is non-malignant clinically and there treated by radiotherapy. Much has been written about this are no recorded deaths from PVNS nor any proved condition since 1941, but we know little more now than that incidents of metastasis. written in this classic paper. Research into PVNS is difficult because of the rarity of the disorder and most series have had very few patients or Clinical features have grouped cases from different anatomical sites. In a rare The classical clinical picture ofPVNS is that of a young man epidemiological study of this condition Myers et al found with monoarticular involvement of the knee with the diffuse the incidence to be 1-8 per million population.4 This is form of the disease. Other leg joints may be involved but probably an underestimate as they looked only at patients virtually always in isolation. The nodular form tends to who had undergone surgery. They found a higher incidence affect the fingers but can occur in the knee, where it may in men, though other investigators have not always found mimic a meniscal lesion.8 The course of the disease is this sex difference. The age range was from 11 to 84, but as insidious with slow progression of symptoms being the rule. with other series young men were most often affected and Discomfort rather than pain is a common complaint and the knee was the most commonly involved joint. They were swelling is invariably present. In patients with longstanding unable to find any clear aetiological associations. disease stiffness is a feature. The cause and pathogenesis of PVNS remain obscure. Routine haematological, biochemical, and immunological Pigmented villonodular synovitis 21

tests of peripheral blood have always failed to disclose any Apparently, the best strategy for the patient with diffuse consistent abnormality.4 9 Aspiration of the knee usually involvement of the knee is to treat them on a symptomatic shows xanthochromic fluid, though on occasions bloody basis, to accept that complete synovectomy is difficult to

fluid may be obtained. Analysis of the synovial fluid from achieve and carries a high morbidity when performed as an Ann Rheum Dis: first published as 10.1136/ard.49.4.210 on 1 April 1990. Downloaded from involved joints has never shown any distinctive changes to open procedure, and to perform a lesser, closed procedure, differentiate PVNS from other causes of an effusion.4 which can easily be repeated as necessary if symptoms are The earliest report on radiological features of PVNS was severe enough. It is to be expected that in the future, with by Lewis in 1947,10 but the most substantial series remains the use of the newly available powered arthroscopy tools, that of Smith and Pugh from the Mayo clinic, who reported that closed synovectomy will be used. This combined with on 38 patients with histologically proved PVNS." In the use of continuous passive motion after operation should patients with moderate involvement soft tissue synovial reduce the morbidity of this procedure. Arthroscopic swelling is seen and the nodular nature of this may give a synovectomy may not be as complete as that performed by clue to the diagnosis. In patients with more advanced an arthrotomy but is of much lower morbidity and can be disease smooth pressure defects develop in the adjacent more readily repeated if necessary. In fact there is some para-articular region, and only in these patients is loss of evidence that PVNS may be a self-limiting condition, with joint space seen with irregularity of the articular surfaces. remission occasionally following biopsy alone. 14 Extra-articular extension and cortical erosion are rare but In other joints apart from the knee similar principles may occur. Calcification is very rare and is more consistent apply. In the hip and shoulder, however, diagnosis is often with a synovial sarcoma. Osteoporosis is surprisingly made late, at the stage when secondary degenerative uncommon even in patients with advanced disease. These changes have already occurred, and the only option then changes are non-specific and plain radiography cannot available is arthrodesis or arthroplasty. These have also been therefore confirm the diagnosis. used as salvage procedures in the knee. As most of these In the past arthrography has been widely used in patients are young, arthrodesis is generally preferred. diagnosis of PVNS and shows filling defects. Johansson et Perhaps the most important factors to bear in mind when al, however, found that arthrography was diagnostic in only treating PVNS are that it is not malignant and should not be one third of cases and in most patients was normal. One treated as such and that early diagnosis and treatment are might hope that newer diagnostic imaging methods might essential to reduce the incidence of recurrence and possibly improve upon this low success rate but to date there have of joint destruction. We can only hope that in the future our been few reports of the use of computed tomographic knowledge will advance further than it has in the last half scanning in PVNS and none ofmagnetic resonance imaging. century since Jaffe's classic account. The latter probably holds most promise for the future. Despite the widespread acceptance of the use of arthro- Royal National Orthopaedic Hospital, GEORGE BENTLEY scopy as a diagnostic tool, in recent years there have been Brockley Hill, Stanmore, surprisingly few reports of the use of the arthroscope in Middlesex HA7 4LP PVNS. 2 3 Obviously, when combined with synovial biopsy it offers the best method of diagnosis. Orthopaedic Department, T McAULIFFE* Whipps Cross Hospital, Leytonstone,

London Eli http://ard.bmj.com/ Treatment In the absence of any clear knowledge of the pathogenesis, *Correspondence to Mr McAuliffe. or of any large prospective studies, treatment of PVNS in the past has been predominantly surgical and largely pragmatic. It is clear that this is a non-malignant disorder 1 Jaffe H L, Lichtenstein L, Sutro C J. Pigmented villonodular synovitis, bursitis and tenosynovitis. Archives ofPathology 1941; 31: 731-65. and therefore radical treatment is not indicated. On the 2 Chassaignac M. Cancer de la gaine des tendons. Gazette Hopital Civil Militaire other hand, suboptimal treatment will lead to recurrence. 1852; 47: 185-6. that 3 Moser E. Primares sarkom der fussgelenkkapsel. Exstirpaton Dauerheilung on October 1, 2021 by guest. Protected copyright. There is general consensus among most authors Deutsche Zeitschrifte fur Chirurgie 1909; 98: 306-10. simple excision of the localised form of the condition is 4 Myers B W, Masi A 1, Feigenbaum S L. Pigmented villonodular synovitis and tenosynovitis. A clinical epidemiological study of 166 cases and adequate, producing good function postoperatively and a literature review. Medicine (Baltimore) 1980; 59: 223-38. low rate ofrecurrence. Treatment ofdiffuse PVNS has been 5 Molnar Z, Stern W H, Stoltzner G H. Cytoplasmic tubular structure in pigmented villonodular synovitis. Arthritis Rheum 1971; 14: 784. less satisfactory. Most authors advocate total synovectomy 6 Hirohata K. Light microscopic and electron microscopic studies of individual in the knee, but even this may not be curative, presumably cells in pigmented villonodular synovitis and bursitis. KobeJ Med Sci 1968; 14: 251-79. owing to the difficulty of performing a truly total syno- 7 Rao A S, Vigorita V J. Pigmented villonodular synovitis (giant cell tumour of vectomy. Johansson reported 33% recurrence after syno- tendon sheath and synovial membrane). A review of 81 cases. J Bone Joint Surg [Am] 1984; 66: 76-94. vectomy in the knee and in the Royal National Orthopaedic 8 Granowitz S P, Mankin H J. Localised pigmented villonodular synovitis of Hospital series it was nearly 50%. 14 In addition, the price to the knee. Report of five cases. J Bone joint Surg [Am] 1967; 49: 122-8. be in terms of function of the knee be as 9 Johansson J E, Ajjouls S, Coughlin L P, Wener J A. Cruess R L. Pigmented paid may high villonodular synovitis of joints. Clin Orthop 1982; 163: 159-66. stiffness and pain are common sequelae after operation. 10 Lewis R W. Roentgen diagnosis of pigmented villonodular synovitis and synovial sarcoma of the knee joint. Preliminary report. Radiology 1947; 49: Radiotherapy has been commonly used in the past, 26-38. mostly for recurrences but also for primary disease.'5 11 Smith J H, Pugh D G. Roentgenographic aspects of articular pigmented villonodular synovitis. AIR 1962; 87: 1146-56. Although there have never been any reported cases of 12 Flandry F C, Jacobson K E, Andrews J R. Localised pigmented villonodular malignancy following irradiation for PVNS, most recent synovitis of the knee mimicking meniscal injury. Arthroscopy 1986; 2: 217-21. commentators have felt that the potential risks of radiation 13 Dorfman H. Arthroscopic detection of synovial disorders. Contemporary treatment were too high in young patients with a benign Orthopedics 1985; 10: 19-29. condition. There may, be in 14 Byers P D, Cotton R E, Deacon 0 W, et al. The diagnosis and treatment of however, a place for it pigmented villonodular synovitis. J Bone Joint Surg [Br] 1968; 50: recurrent, severely symptomatic disease in the elderly. 290-305. Radiation synovectomy 15 Friedmann M, Schwartz E E. Irradiation therapy of pigmented villonodular has been widely used in rheumatoid synovitis. Bulletin of the Hospital for Joint Diseases 1957; 18: 19-32. arthritis,'6 but there is only a solitary case report of its use in 16 Sledge C B, Atcher R W, Shortkroff S, Anderson R J, Bloomer W D, Hurson PVNS. 7 B J. Intra-articular radiation synovectomy. Clin Orthop 1984; 182: 37-40. Potentially it carries the same risks as radiotherapy 17 Wiss D A. Recurrent villonodular synovitis of the knee. Successful treatment and should probably be avoided in the younger patient. with yttrium 90. Clin Orthop 1982; 169: 139-44.