Annals ofthe Rheumatic 1991; 50: 337-339 337

2 Harvey A M, Shulman L E, Tumulty P A, Conley C L, Schoenrich E H. Systemic lupus erythematosus: review of the literature and LETTERS TO clinical analysis of 138 cases. Medicine (Balti- Ann Rheum Dis: first published as 10.1136/ard.50.5.337-b on 1 May 1991. Downloaded from more) 1954; 33: 291-437. 3 Sharp G C, Anderson P C. Current concepts in THE EDITOR the classification of connective tissue . J Am Acad Dermatol 1980; 2: 269-79. 4 Fox R, Rosahn P. The lymph nodes in dis- seminated lupus erythematosus. Am J Pathol 1943; 19: 73-95. 'Lupus lymphadenitis' simulating a 5 Ginzler A M, Fox T T. Disseminated lupus erythematosus: a cutaneous manifestation of a strangulated femoral hernia in a patient with systemic disease (Libman-Sachs). Report of a mixed connective tissue disease case. Arch Intern Med 1940; 65: 26-50. 6 Klemperer P, Pollack A D, Baehr C. Sir: Lymphadenopathy is a common finding of disseminated lupus erythematosus. Arch Pathol Lab Med 1941; 32: 569-631. in both systemic lupus erythematosus (SLE) 7 Strickler J G, Wamke R A, Weiss L M. and mixed connective tissue disease (MCTD)' in lymph nodes. PatholAnnu 1987; 22: 253-82. and is often associated with disease activity. 8 Bennett R M, O'Connell D J. Mixed connective tissue disease; a clinicopathologic study of 20 Although lymph nodes may be tender and the cases. Semin Arthritis Rheum 1980; 10: 25-51. degree of lymphadenopathy sufficient to cause 9 Lewis R B, Castor C W, Kinsley R E, Bole G G. concern over the possibility of primary Frequency of neoplasia in systemic lupus lymphatic disease,2 an acute presentation with erythematosus and rheumatoid arthritis. Arthnrtis Rheum 1976; 19: 1256-60. localised lymphadenopathy in the absence of 10 Miller D G. The association of immune disease other systemic features is exceptional. We and malignant lymphoma. Ann Intern Med report a patient in whom the clinical presenta- 1%7; 66: 507-21. 11 Kassan S S, Thomas T L, Moutsopoulos H M, et tion suggested the diagnosis of a strangulated al. Increased risk of lymphoma in sicca syn- femoral hernia, but in whom the true diag- Section of lymph node shounng marked necrosis. drome. Ann Intern Med 1978; 89: 888-92. nosis, proved histologically, was lupus (Haematoxylin and eosin.) 12 Rosenstein E D, Wieczorek R, Raphael B G, lymphadenitis. Agus B. Systemic lupus erythematosus and angioimmunoblastic lymphadenopathy: case A 25 year old woman presented with a five report and review of the literature. Semin day history of a painful lump in her right groin nodes, suggesting more generalised involve- Arthn'tis Rheum 1986; 16: 146-51. associated with a raised temperature and ment. The pathology of lymph nodes in SLE general malaise. She had had some transient is well reported and characteristic, 46 though swelling in her right groin five months earlier. the remains unknown.' Affected In 1983 she had had a partial thyroidectomy lymph nodes show a necrotising lymphadenitis Raised concentrations of von Willebrand for thyrotoxicosis and in 1986 she was diag- with lymphoblastic and immunoblastic factor antigen in systemic sclerosis nosed as having SLE when she presented with responses. Advanced lesions may also show general malaise, weight loss, Raynaud's haematoxylin bodies and deposits of haemat- Sir: von Willebrand factor antigen is the phenomenon, and a cough. She was found to oxyphilic material in the walls of blood vessels antigenic component ofvon Willebrand factor, have diffuse hypergammaglobulinaemia, a (the Azzopardi phenomenon) with perivascular and circulating concentrations are raised in negative rheumatoid factor, a positive anti- .7 vasculitis and systemic sclerosis, reflecting nuclear factor (titre 1/80, speckled pattern), There are few reports of the pathology of damage to endothelial cells.' 2 In systemic positive anti-DNA binding (titre 1/2560), and lymph nodes in MCTD. Most are said to show sclerosis concentrations of von Willebrand a positive extractable nuclear antigen (titre non-specific changes.' Two cases have been factor antigen correlate with a risk ofmortality' 1/732). She then had two episodes of myo- described, however, in which necrosis was and with the extent of visceral disease-that is, pericarditis, both of which responded well to associated with lymphoid .8 In one involvement of heart, lung, muscle, etc.4 http://ard.bmj.com/ intravenous methylprednisolone. She has now of these the necrosis was associated with Neither of these reports directly considered developed sclerodermatous changes affecting thrombosis, resulting in hilar infarction. the question of concentrations of von Wille- her hands and face and oesophageal dysmo- Other types of lymphadenopathy may also brand factor antigen in the two major sub- tility. She has never had any clinical features be found in patients with connective tissue groups of systemic sclerosis-the limited to suggest Sjogren's syndrome. Her auto- disease. Malignancy may occur more com- cutaneous variant (CREST) and the diffuse antibody pattern has changed with the de- monly in patients with SLE, particularly variant. velopment of antiribonucleoprotein antibodies those receiving immunosuppressive drugs.9 We therefore studied 14 patients with and the loss of DNA binding, suggesting Lymphoproliferative disorders have been clearly defined CREST but no proximal skin on September 27, 2021 by guest. Protected copyright. progression to MCTD with features of sclero- reported to be associated with an increased disease and six with the clearly defined diffuse derma. Throughout this time her maintenance incidence of rheumatoid arthritis compared variant with proximal scleroderma,5 and com- dose of oral prednisolone and azathioprine has with a control population'0 and to occur pared them with 20 apparently healthy labora- been continued. On examination she was 44 times more commonly in patients with tory and hospital staff as controls matched for cushingoid with facial telangiectasia, micro- Sjogren's syndrome. " Furthermore, angio- age and sex. von Willebrand factor antigen in stomia, and sclerodermatous changes in her immunoblastic lymphadenopathy, a non- plasma was measured by a standard enzyme hands. She was afebrile, but had a tender mass neoplastic proliferative disorder of lympho- linked immunosorbent assay (ELISA).6 in her right groin measuring 3x3 cm, which cytes, shows many clinical and laboratory Results are shown in the figure. von Wille- was thought to be a strangulated femoral similarities to SLE and may be associated with brand factor antigen was raised in patients hernia. At operation she was found to have .12 with systemic sclerosis relative to normal several enlarged lymph nodes below the right In conclusion, we know of no previous controls (median 1500 IU/I, controls 800 IU/I, inguinal ligament. She made a good post- report of lupus lymphadenitis simulating a p

relate anticardiolipin antibodies with Pneumo- 5 Canoso R T, Zon L I, Groopman J E. Anticardio- 30001 lipin antibodies associated with HTLV-III Cystis carinii ,3 other opporniic infection. BrJ Haematol 1987; 65: 495-8. Ann Rheum Dis: first published as 10.1136/ard.50.5.337-b on 1 May 1991. Downloaded from , and with ,4 but up to the 6 Mizutani W T, Woods V L, McCutchan J A, present, anticardiolipin antibodies in patients Zvaifler N J. Anticardiolipin antibodies in with AIDS have only been associated with a human immunodeficiency virus (HIV) infected poor outcome of the illness.5 6 In gay males may be associated with a deteriorat- these ing clinical course. Arthritis Rheum 1987; 30: 0) patients anticardiolipin antibodies do not have S35. 0) the same clinical significance as they have in systemic lupus erythematosus.' 0 0 As drug addicted patients with AIDS have a Disabling ossification of the pateliar tendon ~0 different spectrum of rheumatic manifesta- .0. 0 0 tions, with less reactive arthritis, Reiter's Sir: A 42 year old lorry driver without any

S syndrome, and HIV related arthritis, 0 and medical or traumatic history had had for two .0 more septic arthritis than homosexual patients years pains in the right knee when walking. =1000- -IF with AIDS (Monteagudo I et al, unpublished They had appeared simultaneously with the data), westudiedthepresenceofanticardiolipin use of a new 0 truck, in which the accelerator antibody in drug addicted patients with AIDS pedal was particularly stiff. After some months and its relation with clinical manifestations. of limping clinical examination showed that Anticardiolipin antibody was determined the right patellar tendon was diffusely thick- (Cheshire Diagnostic QACA enzyme linked ened and tender; a bulge sign was found, as immunosorbent assay (ELISA) kit) in 55 drug well as of the quadriceps muscle and a oj addicted patients. Forty three of these had marked reduction of the knee flexion. Diffuse CREST Ery- been admitted to hospital because of acute throcyte sedimentation rate was 1 mm/i st h; Controls Systemic sclerosis problems related to AIDS (mainly infections) fasting blood sugar, calcium, phosphorus, and and the remaining 12, without acute problems, alkaline phosphatase were normal. Synovial Plasma von Wilebrandfactor antigen were seen at the outpatient AIDS clinic in the fluid x concentrations. Each point represents one patient contained 0 1 I09 leucocytes/l without or control. The bar is the median value. hospital. Anticardiolipin antibodies were posi- crystals. tive in 42 (76%)-31/43 (72%) of the hospital Lateral plain radiographs and tomograms patients and 11/12 (92%) of the outpatients. (without signs of patella alta: length of the of a higher von Willebrand factor antigen in Clinical problems in the hospital patients were patellar tendon equal to the diagonal length of patients with diffuse disease suggests endothe- diverse, and we found no significant relation the patella') showed ossification of the tendon, lial may be more extensive and between infection and the presence of anti- which did not affect its distal third (fig 1). active in this group. The extent of vascular cardiolipin antibodies. Additionally, there was Sonography showed that ossification was injury as shown by von Willebrand factor no relation between anticardiolipin antibodies mostly in the lateral part and that the non- antigen concentrations may be regarded as a and the antecedent and kind of infection in ossified distal third was thickened in com- prognostic marker in these disorders. these patients. Of the 55 patients, 43 were in parison with the tendon on the left side stage IV AIDS, with 34 (79%) being positive (anteroposterior thickness 9 mm v 6 mm). A D BLANN for anticardiolipin antibodies. The remainder Radiographs and computed tomography also Department ofSurgery were in other stages and anticardiolipin anti- showed irregular enthesitic osteophytes of the Research and Teaching Building bodies were found in 10/12 (83%). upper part of on both knees in University Hospital ofSouth Manchester patellae and, Nell Lane, Didsbury Our results show that the presence of addition, a femoral and tibial osteopoikilosis. Manchester M20 8LR, UK anticardiolipin antibodies in drug addicted Radiographs of cervical, thoracic, and lumbar patients with AIDS is similar to that found in spine showed no abnormalities. KAREN J ILLINGWORTH homosexual patients with AIDS. We found no http://ard.bmj.com/ M I V JAYSON Arthroscopic examination showed the joint University ofManchester Rheumatic Diseases Centre relation between anticardiolipin antibodies cavity was normal. During operation the Clinical Sciences Building and clinical manifestations (mainly infections) orthopaedic surgeon found that the right Hope Hospital nor pathological antecedents in these patients. patellar tendon was wider than normal. The Eccles Old Road Similarly, there were no differences Salford, Manchester M6 8HD, UK between ossified mass was not adherent to the adjacent outpatients and hospital patients. As with patella or tibia and could be easily dissected homosexual patients with AIDS, we found no and removed. The patient recovered slowly correlation between anticardiolipin antibodies I Belch J J F, Zoma A A, Richards I M, and was able to resume his work three months McLaughlin K, Forbes C D, Sturrock R D. and recurrent thrombosis and thrombopenia. later. on September 27, 2021 by guest. Protected copyright. Vascular damage and factor-VIII-related anti- Probably, anticardiolipin antibodies are as- Pathological examination showed compact gen in the rheumatic diseases. Rheumatol Int sociated with HIV infection itself or with an remodelled lamellar bone. In the mass of the 1987; 7: 107-1 1. 2 Kahalch M B, Osborn I, Leroy E C. Increased abnormal immune response which is not yet bone, and on the proximal end, strips of factor VIII/von Willebrand factor antigen and well defined. fibrous cartilage were seen, which might be von Willebrand factor activity in scleroderma considered as an abnormal of the and in Raynaud's phenomenon. Ann Intern JAVIER RIVERA tendon (figs 2A and B). Despite its distance Med 1981; 94: 482-4. INDALECIO MONTEAGUDO 3 Sheeran T P, Blann A D, Emery P, Bacon P A. JAVIER LOPEZ-LONGO from any insertion site this intermingling of Factor VIII:RAg in primary Raynaud's syn- EDUARDO MALDONADO bone and fibrous cartilage resembles that seen drome and scleroderma. Is it of diagnostic or LUIS CARRENO in enthesopathic hyperostosis-that is, in a prognostic value? Arthritis Rheum 1990; 33 Servicio de Reumatologia location in which fibrocartilaginous bundles (suppl:) S157. Hospital (General Gregorio Marazon 4 Greaves M, Malia R G, Milford Ward A, et al. Madrid, Spain are normally found.2 On the distal end Elevated von Willebrand factor antigen in tendinous bundles intermingled with scar systemic sclerosis: relationship to visceral Correspondence to: Dr J Rivera, Servicio de Reu- tissue associated with sonme degree of scar disease. BrJ Rheumatol 1988; 27: 281-5. matologia, Hospital Gregorio Marauion, Doctor S LeRoy E, Black C, Fleischmajer R, et al. Esquerdo 46, 28007 Madrid, Spain. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rhewmatol 1988; 15: 202-5. 6 Short P E, Williams C E, Picken A M, Hill F G H. Factor VIII related antigen: an improved enzyme immunoassay. Med Lab Sci 1982; 39: 1 Stimmler M M, Quismorio F P, McGehee W G, 351-5. Boylen T, Sharma 0 P. Anticardiolipin anti- bodies in acquired immunodeficiency syn- drome. Arch Intern Med 1989; 149: 1833-5. 2 Bloom E J, Abrams D I, Rodgers G. Lupus Anticardiofipin antibodies in drug addicted anticoagulant intheacquired immnunodeficiency syndrome. JAMA 1986; 256: 491-3. patients with AIDS 3 Gold J E, Haubenstock A, Zalusky R. Lupus anticoagulant and AIDS. N Engl J Med 1986; Sir: The presence ofanticardiolipin antibodies 314: 1252-3. I and 4 Cohen A J, Phillips T E, Kessler G M. Circulat- lupus anticoagulant has been described in ing coagulation inhibitors in the acquired up to 90% of homosexual patients with immunodeficiency syndrome. Ann Intern Med AIDS.' 2 There have been some attempts to 1986; 104: 175-80. Figure I (A) Plain radiograph; (B) tomogram.