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SYSTEMIC LUPUS ERYTHEMATOSUS AND AMYLOIDOSIS SUSANA WEBB, MD, FERNANDO SEGURA, MD, FRANCISCO CERVANTES, MD, ALEJANDRO DARNELL, MD, ELADIO SORIANO, MD, MANUEL RIBAS-MUNDO, MD, and JUAN GARCIA-SAN MIGUEL, MD

Amyloidosis can occur in association with a series of other processes, including connective tissue disease; amyloid deposition has been found in rheumatoid arthritis, dermatomyositis, scleroderma, and periarteritis nodosum. But among the many cases published only four authors describe systemic lupus erythematosus (SLE) and amyloidosis (1-4). We report a case of association of SLE and amyloidosis in a patient in whom radiologic and serologic findings compatible with ankylosing spondylitis were discovered. Case report. JFR, a 62-year-old white woman, was admitted to the hospital with acute pyelonephritis. Her history was not significant except for isolated episodes of noninflammatory arthralgia of the large joints with morning stiffness for the last 25 years, occasional Raynaud’s syndrome of both hands, and more recently, mild congestive heart failure. Physical examination on admission revealed mild congestive heart failure and tenderness on deep pressure in both costovertebral areas; blood-pressure was 130/70 mm Hg, pulse 85 and regular, temperature 38.4OC; chest x-ray film showed basal platelike atelectasis, diaphragmatic elevation, and a widened left costophrenic angle. Hemoglobin was 6.5 gm/ 100 ml; there was anisopoikilocytosis; WBC was 10,100; differential showed 3 1% stab neutrophils; sedimentation rate was 83 mm/hour; serum iron was 18 pg/ 100 ml and iron-binding saturation 13%. BUN was 49 mg/100 ml, creatinine 2.1 mg/100 ml with an endogenous creatinine clearance of 23.6 ml/minute; urine sediment showed 50 leukocytes and 5 erythrocytes per high power field. Electrolytes, fasting blood sugar, SGOT, From the Hospital Clinico, University of Barcelona, Barcelona, Spain. Address reprint requests to Susana Webb, MD, Clinica Medica “B’,Hospital Clinico, Facultad de Medicina, Casanova, 143, Barcelona, Spain. Submitted for publication March 15, 1978; accepted December 18. 1978 Arthritis and Rheumatism, Vol. 22, No. 5 (May 1979)

SGPT, LDH, lipids, bilirubin, coagulation tests, ECG, barium meal and enema, and an intravenous pyelogram were normal. Serum protein electrophoresis showed a broad-based increase in the gammaglobulin (22 gm/ liter) and a decrease in the albumin (21 gm/liter) region; immunoglobulin A and M were normal, but IgG was 2560 mg/100 ml (normal: 600-1500 mg/100 ml); proteinuria oscillated between 1960 and 7530 mg/liter. Blood and urine cultures were positive for E coli and treatment with trimethoprine-sulfametoxazolewas begun. A bone marrow examination showed a decreased red cell series, 1% sideroblasts, and decreased reticuloendothelial iron; a search for occult bleeding from the gastrointestinal tract was slightly positive once and negative on two occasions; we were unable to explain further the cause of this iron deficient anemia. Antinuclear antibodies were positive at 1/2000 and exhibited a speckled pattern; anti-DNA antibodies were positive at 1/200 (by radioimmunoassay); rheumatoid factor, cryoglobulins, and the LE-cell phenomenon were positive; syphilis serologic tests, cryofibrinogen, Coombs test, and antimuscle antibodies were negative; serum complement was reduced to a C’3 of 32 mg/100 ml (normal: 80-140), C’4 of 8 mg/100 ml (normal 2050) and CH50 of 100 units (normal: 400-700). A kidney biopsy revealed by optic microscopy extensive renal amyloidosis and lesions suggestive of necrotizing arteritis; glomerular amyloid material was confirmed in Congo red preparations and amyloid fibrils were also present on electron microscopy; immunofluorescence of the biopsy specimen revealed segmental deposits of C l q , C’4, C’3, and IgG along the glomerular basement membrane (Figure 1). A rectal biopsy also showed amyloid deposits. The patient was discharged on a regimen of 45 mg of prednisone daily by mouth; 2 weeks later she was readmitted because of extensive edema of ankles and feet; proteinuria continued at 2-5 gm/liter; serum pro-

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Figure 1. Kidney biopsy: glomerulus showing extensive involvement by amyloid and reduction in the number of endocapillary nuclei. (H & E; X 160.)

teins had descended to 38 gm/liter of which 15.5% was albumin; a diet rich in proteins was begun and prednisone was increased to 60 mg daily. Although she never complained of sacroiliac pain, and physical examination of the sacroiliac joints showed negative results, x-rays and tomograms (Figure 2) of these joints indicated a bilateral sacroiliitis; histocompatibility typing showed an HLA-B27 antigen. Her general condition deteriorated and she finally died of sepsis with positive urine culture to Salmonella group B and a gram negative anaerobic bacillus of the bacteroides type. Permission for an autopsy was not granted. Discussion. The association of SLE and amyloidosis is rare. It is mentioned by Robbins (1) and Miessmahl(2), and both Wegelius (3) and Schleissner et a1 (4) have published a case report. Our patient had amyloid deposits in the kidney and rectal mucosa; she also fulfilled the ARA criteria for the classification of SLE (51, exhibiting a typical renal biopsy of lupus nephropathy and more than the

Figure 2. Sacroiliac tomogram: bilateral sclerosis and erosions on both sacral and iliac articular surfaces. Bilateral sacroiliitis in phase 2.

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four necessary diagnostic criteria (Raynaud’s phenomenon, arthritis without deformity, LE cells, profuse proteinuria, cellular casts, pleuritis as well as reduced serum complement and positive ANA test, two points worthy to be considered as criteria according to Dubois) (5). Given the rarity of this association, we decided to look for other possible causes of amyloidosis. All the results of the investigations carried out in this respect were negative except the sacroiliac x-rays, which demonstrated a clinically asymptomatic bilateral sacroiliitis, and the positive B27 histocompatibility antigen. Although our patient fulfilled only one (sacroiliac involvement) of the internationally defined criteria for the diagnosis of ankylosing spondylitis, the presence of B27 makes this diagnosis possible, since according to Leca et a1 ( 6 ) this antigen is present in more than 90% of cases of ankylosing spondylitis and should therefore be considered a major criterion. Studies on histocompatibility antigens have been carried out in SLE (7), but no differences have been found between the incidence of positive HLA-B27 antigen in SLE and a control group (8). We must agree then that our patient probably suffered from ankylosing spondylitis. The association of ankylosing spondylitis and amyloidosis is well documented (9-14), but the association of systemic lupus erythematosus, ankylosing spondylitis, and amyloidosis has not been described. Acknowledgments. We wish to acknowledge the helpful suggestions and encouragement of Professor Alan S . Cohen of Boston City Hospital, Boston, Massachussetts.

REFERENCES 1. Robbins SL: Amyloidosis, Pathologic Basis of Disease.

Edited by SL Robbins. Philadelphia, WB Saunders Company, 1974, pp 281-290 2. Miessmahl JP: Symposium on amyloidosis. Part 1. Edited

by E Mandema. Amsterdam, Excerpta Medica Foundation, 1967, pp 22-33 3. Wegelius 0: Amyloidosis of the kidneys, adrenals and spleen as a complication of acute disseminated lupus erythematosus treated with ACTH and cortisone. Acta Med Scand 156:9 1-95, 1956 4. Schleissner LA, Sheehan WW, Orselli RC: Lupus erythematosus in a patient with amyloidosis, adrenal insufficiency, and subsequent immunoblastic sarcoma. Demonstration of the LE phenomenon in the lung. Arthritis Rheum 19:249%255,1976 5. Dubois EL: Differential diagnosis, criteria for diagnosis, and classification for systemic lupus erythematosus, Lupus Erythematosus. Edited by EL Dubois. Los Angeles, University of Southern California Press, 1974, pp 534-536 6. Leca AP, Crouzet J, Attali P, Prier A, Camus JP: IntCret de la recherche du groupe HL-A W 27 pour le diagnostic des formes frustes ou atypiques de spondylarthrite ankylosante sans signes radiologiques. Ann Med Interne (Paris) 125:755-757, 1974 7. Suciu-Foca N, Buda J, Almojera P, Reemtsma K: HL-A antigens and M.L.C. responsiveness in systemic lupus erythematosus. Lancet 2:726-727, 1974 8. Nies KM, Brown JC, Dubois EL, Quismorio FP, Friou GJ, Terasaki PI: Histocompatibility (HL-A) antigens and lymphocytotoxic antibodies in systemic lupus erythematosus (SLE). Arthritis Rheum 17:397402, 1974 9. Benedek TG, Zawadzki ZA: Ankylosing spondylitis with ulcerative colitis and amyloidosis: report of a case and review of the literature. Am J Med 40:431439, 1966 10. Cruickshank B: Pathology of ankylosing spondylitis. Bull Rheum Dis 10:211-214, 1960 11. Leading articles and annotations: Amyloidosis and ankylosing spondylitis. Jr Assoc Phys Ind 23:382-383, 1975 12. Tiku ML, Mathai N, Chakrabarti AK, Johnson SC, Bhaktaviziam A: Amyloidosis complicating ankylosing spondylitis. J Assoc Phys Ind 23:393-396, 1975 13. Somer T, Siltanen P: Aneurysm of descending aorta, amyloidosis and renal carcinoma in a patient with ankylosing spondylitis. Am J Med 49:408415, 1970 14. Bywaters EGL: A case of early ankylosing spondylitis with fatal secondary amyloidosis. Br Med J 2: 412416, I968

Systemic lupus erythematosus and amyloidosis.

5 54 BRIEF REPORTS SYSTEMIC LUPUS ERYTHEMATOSUS AND AMYLOIDOSIS SUSANA WEBB, MD, FERNANDO SEGURA, MD, FRANCISCO CERVANTES, MD, ALEJANDRO DARNELL, MD...
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