Annals ofthe Rheumatic Diseases 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 disease. 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. Pathology 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. Necrosis 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 pathogenesis 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- inflammation.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 hyperplasia.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<OOI, Mann-Whitney U test used through- operative recovery and has since remained surgical emergency. This case also underlines out). Analysis by subgroup showed that both well with her usual maintenance dose of the fact that ng clear pathological distinction the diffuse variant (1770 IU/l, p<OOI) and prednisolone 7 mg/day and azathioprine 50 can be drawn between SLE and MCTD. the CREST variant (1150 IU/l, p<005) had mg/day. Histology of the lymph nodes was raised concentrations of von Willebrand factor characteristic of lupus lymphadenitis showing N j SIMMONDS antigen, which were higher in the patients necrosis with nuclear debris under- B I HOFFBRAND with the diffuse variant than in those with the (figure) Whittington Hospital going phagocytosis in one lymph node and Highgate Hulll CREST variant (p<O0OI). both centroblastic and immunoblastic London N19 SNF These data confirm and extend previous responses in adjacent nodes. reports about von Willebrand factor antigen in Correspondence to: Dr N J Simmonds, Gastro- systemic sclerosis. Sheeran et a! studied 34 Localised or generalised lymphadenopathy intestinal Science Research Unit, 26 Ashfield Street, occurs in about half of patients with SLE at London El 2AJ. patients with systemic sclerosis, of whom some stage during their disease' and in about a seven died (mean von Willebrand factor anti- third of patients with MCTD.3 In SLE super- gen 2980 IU/l, in the surviving group 1450 ficial nodes, particularly those in the neck, are IU/1).3 Greaves et al found a mean von I Paradinas F. and secondary immune 4 Primary Willebrand factor of 1730 U/l in 16 commonly affected. Necropsy studies, how- disorders. In: Stansfeld A G, ed. Lymph node antigen ever, commonly show enlargement of mesen- biopsy interpretation. Edinburgh: Churchill patients with severe disease and 1030 U/I in 12 teric, tracheobronchial, and retroperitoneal Livingstone, 1985: 159-95. patients with mild disease.4 Our finding 338 Letters to the editor 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 infection,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 infections, and with neoplasm,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.