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.