Annals ofthe Rheumatic Diseases 1996; 55: 421-422 421

LESSON OF THE MONTH Ann Rheum Dis: first published as 10.1136/ard.55.7.421 on 1 July 1996. Downloaded from Systemic erythematosus: an occasional misdiagnosis

D A Collins, B E Bourke

Case report phosphatase 917 IU/l (NR 30-100 IU/l), and A 54 year old West Indian woman with y-glutamyltransferase 836 IU/l (NR 0-30 IU/ hypertension and diet controlled diabetes 1)), and mild hypercalcaemia (corrected mellitus was admitted with a six week history calcium 2-65 mmol/l (NR 2-15-2-55 mmol/1)). of general , fever, mouth ulceration, Her ANA remained negative. A liver myalgia, arthralgia, and arthritis; previously specimen revealed multiple non-caseating she had been well. On examination she was granulomata (figure) and her diagnosis was noted to be feverish (38°C) with bed changed to . Angiotensin converting infarction, splinter haemorrhages, and mouth enzyme concentration was subsequently found ulceration, but no or lymphadenopathy. to be 284 IU/l (NV <105 IU/1); repeat chest There was generalised muscle and joint radiography showed no evidence of or tenderness, with synovitis of the knees and hilar disease. Her steroids were increased, ankles. Investigations revealed a normal azathioprine was added to her treatment, and haemoglobin concentration and platelet count, currently she remains well. lymphopenia (1000/mm3; normal value (NV) >1 500/mm3), erythrocyte sedimentation rate Discussion (ESR) 59 mmllst h, and C reactive protein SLE and sarcoidosis share many clinical and (CRP) 460 mg/l (NV <80 mgfl); creatine immunological features, including hyperglobu- kinase, renal and liver function tests, linaemia, decreased lymphoid responsiveness, electrocardiogram, and were and increased circulating immune complexes normal. Echocardiogram revealed a moderate (table). Positive ANA is reported in association pericardial effusion, but no evidence of with sarcoidosis in up to 33% of patients endocarditis, and screening for infection was during the acute phase,1 decreasing to the same http://ard.bmj.com/ negative. frequency as in controls if the disease becomes A provisional diagnosis ofa connective tissue inactive.2 Antibodies to double stranded DNA disease was made and, whilst serological tests have not been reported. were pending, the patient was discharged from Arthritis, bilateral hilar lymphadenopathy, hospital with non-steroidal anti-inflammatory and nodosum (Lofgren's syndrome) medication. However she was readmitted with is perhaps the most common presentation of worsening joint pains, pleurisy, and a sarcoidosis seen by the rheumatologist, but on September 30, 2021 by guest. Protected copyright. widespread urticarial rash. Further investiga- fevers, arthritis, mouth ulceration, and pleuro- tions revealed her to be antinuclear antibody pericarditis have all been described, both in (ANA) positive (homogeneous pattern, titre early and in active chronic sarcoidosis, together not stated) and anticardiolipin antibody with non-specific and serological positive, but anti-DNA antibody negative. evidence of immune system activation. Complement and immunoglobulin concentra- Neither nor hilar tions were increased; rheumatoid factor, lymphadenopathy was present in the patient hepatitis serology, and Coomb's test were described, and with her clinical presentation negative. A biopsy specimen from involved, and immunological abnormalities she fulfilled non-light-exposed skin showed non-specific five of the 11 ARA classification criteria for inflammatory changes with no lupus band on SLE.' CRP is not reliably decreased in SLE immunofluorescence. A diagnosis of systemic and the increased CRP concentration was felt (SLE) was made, and her to be compatible with her disease. The lupus Department of treatment drug was changed to prednisolone band test is positive in only 50% of cases in , 20 mg daily. uninvolved non-light-exposed skin, increasing St George's Hospital, London SW17 OQT, The patient's condition rapidly improved to 90% in involved light exposed skin, and United Kingdom and she was able to return to work; her ANA negative lupus band does not exclude the D A Collins became negative and the steroid dosage was diagnosis. The disappearance of the ANA with BE Bourke gradually reduced to a maintenance level of persisting increase in ESR and deranged liver Correspondence to: Dr D A Collins, 5 mg daily. However, she continued to com- function were atypical, however, and led to Department of plain ofgeneral malaise and her ESR remained revietv of the initial diagnosis. Rheumatology, Princess Margaret Hospital, persistently >50 mi/lst h. Coexistent SLE with sarcoidosis is rare, with Okus Road, Twenty months after first presentation there only five cases reported in the English Swindon SN I 4JU, United Kingdom. was a marked deterioration in the patient's liver literature,' three ofwhich4 7 8 showed features Accepted for publication function (serum alanine aminotransferase 72 similar to those ofthe patient we describe, with 1 February 1996 IU/l (normal range (NR) 5-40 IU/1), alkaline early features giving a diagnosis of SLE, but 422 Collins, Bourke

coexistent disease or mislabelling is not clear, and the frequency of the association of sarcoidosis and SLE remains unknown. Ann Rheum Dis: first published as 10.1136/ard.55.7.421 on 1 July 1996. Downloaded from It would appear that the early immuno- logical reactions of sarcoidosis may be mistaken for SLE, and this should be con- sidered in the differential diagnosis and when the diagnosis is re-evaluated. The lesson * Sarcoidosis may mimic the clinical presentation of SLE, and fulfil the diagnostic criteria. * Sarcoidosis should be considered in the differential diagnosis of SLE, both in the early stage and during long term follow up.

The authors are grateful to Dr Caroline Finlayson for providing the photomicrograph. Liver biopsy specimien showing non-caseating granuloniata. Reticulin stain; original magnification X 250. 1 Veien N K, Hardt F, Bendixen G, et al. Immunological studies in sarcoidosis: a companson of disease activitv and Similarities and differences in the investigation ofsvstemnc lupus erythematosus (SLE) anid various immunological parameters. Amn NY Acad Sci sarcoidosis 1976; 278: 47-51. 2 Olenchock S A, Marx J J, Mull J C, et al. Immunology of SLE Sarcoidosis clinically inactive sarcoidosis: serum factors. Annpi Allergo' 1981;47:28-31. Chest radiograph 3 Tan E M, Cohen A S, Fries J F, et al. The 1982 revised Mediastinal lvmphadenopathv May be present Frequently present critena for the classification of systemic lupus (early disease) erythematosus. Arthritis Rheumi 1982; 25: 1271 -7. Interstitial lung disease Maxy be present Common (late disease) 4 Harrison G N, Lipham M, Elguindi A S, Lobel D H. Acute ANA +ve 95- 1000/o +ve 5-30°O sarcoidosis occurring during the course of systemic lupus Specific antibodies Frequently present Not reported erythematosus. South Med7 1979; 72: 1387-8. (DNA, ENA) 5 Wiesenhutter C W, Sharma 0 P. Is sarcoidosis an Hyperglobulinaemia Present Present ? Report of 4 cases and review of the Hypocomplementaemia Common Not reported literature. Seniun Arthritis Rheum 1979; 9: 124-40. Immune complexes Frequently present 25-75% (with EN) 6 Hunter T, Amott J E, McCarthv D S. Features of systemic Hypercalcaemia Rare Frequently present lupus erythematosus and sarcoidosis occurring together. ACE Normal Frequently increased Arthritis Rheum 1980; 23: 364-6. Kveim test Negative Positive 7 Needleman S W, Silber R A, Von Brechv J H, Goeken J A. Systemic lupus erythematosus complicated bv dis- ACE = Angiotensin converting enzvme; ANA = antinuclear antibodv; EN = erythema nodosum, seminated sarcoidosis. Ami73 Cli/r Pathol 1982; 78: 105-7. ENA = extractable nuclear antigen. 8 Hammond J M J, Bateman E D. Successful treatment of life-threatening steroid resistant pulmonary sarcoidosis http://ard.bmj.com/ with cvclosporin in a patient with svstemic lupus erythematosus. Respir Med 1990; 84: 77-80. 9 Soto-Aguilar M C, Boulware D W. Sarcoidosis presenting development of sarcoidosis within 18 months. as antinuclear antibody positive glomerulonephritis. Ann In addition, Soto-Aguilar et al reported a Rheumi Dis 1988; 47: 337-9. in whom sarcoid 10 Teilum G. Miliary epithelioid-cell granulomas in lupus patient glomerulonephritis ervthematosus disseminatus. Acta Pathol Mlicrobi()l Scanid was initially mistaken for SLE.9 Non-caseating 1945; 22: 73-9. 11 Pollak A D. Some observations on the pathologs of systemic granulomas were reported in early studies of lupus ervthematosus. Moioit Sinai Hosp XIY 1959; _7 26: on September 30, 2021 by guest. Protected copyright. SLE,i1 ii though whether this represents 224-40.