Restricted accessResearch articleFirst published online 2008-7
Systemic lupus erythematosus and familial Mediterranean fever: a possible negative association between the two disease entities – report of four cases and review of the literature
Serositis is a common clinical manifestation of systemic lupus erythematosus (SLE), as well as being the hallmark of familial Mediterranean fever (FMF), the most prevalent monogenic disease in the Jewish population. We have treated four patients who suffered from both SLE and FMF since 2001 in our clinic, which also serves as the national center for FMF. Our cases illustrate both similarities and dissimilarities between the clinical manifestations of these two diseases, an aspect which should be borne in mind, especially in the young female patients. In general, it seems that co-occurrence of FMF moderates the presentation of lupus.
1 Gaubitz M . Epidemiology of connective tissue disorders.Rheumatology (Oxford)2006; 45(Suppl. 3): iii3 – iii4 .
2.
2 Tan EM Cohen AS Fries JF . The 1982 revised criteria for the classification of systemic lupus erythematosus.Arthritis Rheum1982; 25: 1271 – 1277 .
3.
3 Sohar E Gafni J Pras M Heller H . Familial Mediterranean fever. A survey of 470 cases and review of the literature.Am J Med1967; 43: 227 – 253 .
4.
4 Kogan A Shinar Y Lidar M . Common MEFV mutations among jewish ethnic groups in israel: high frequency of carrier and phenotype III states and absence of a perceptible biological advantage for the carrier state.Am J Med Genet2001; 102: 272 – 276 .
5.
5 Fidder H Chowers Y Ackerman Z . The familial Mediterranean fever (MEVF) gene as a modifier of crohn's disease.Am J Gastroenterol2005; 100: 338 – 343 .
6.
6 Balaban B Yasar E Ozgul A Dincer K Kalyon TA . Sacroiliitis in familial Mediterranean fever and seronegative spondyloarthropathy: importance of differential diagnosis.Rheumatol Int2005; 25: 641 – 644 .
7.
7 Langevitz P Livneh A Zemer D Shemer J Pras M . Seronegative spondyloarthropathy in familial Mediterranean fever.Semin Arthritis Rheum1997; 27: 67 – 72 .
8.
8 Schwartz T Langevitz P Zemer D Gazit E Pras M Livneh A . Behcet's disease in familial Mediterranean fever: characterization of the association between the two diseases.Semin Arthritis Rheum2000; 29: 286 – 295 .
9.
9 Schlesinger M Rubinow A Vardy PA . Henoch-schonlein purpura and familial Mediterranean fever.Isr J Med Sci1985; 21: 83 – 85 .
10.
10 Gur H Tchakmakjian L Eherenfeld M Sidi Y . Polyarteritis nodosa: a report from israel.Am J Med Sci1999; 317: 238 – 242 .
11.
11 Glikson M Galun E Schlesinger M . Polyarteritis nodosa and familial Mediterranean fever: a report of 2 cases and review of the literature.J Rheumatol1989; 16: 536 – 539 .
12.
12 Langevitz P Zemer D Livneh A Shemer J Pras M . Protracted febrile myalgia in patients with familial Mediterranean fever.J Rheumatol1994; 21: 1708 – 1709 .
13.
13 Rabinovich E Livneh A Langevitz P . Severe disease in patients with rheumatoid arthritis carrying a mutation in the Mediterranean fever gene.Ann Rheum Dis2005; 64: 1009 – 1014 .
14.
14 Rozenbaum M Rosner I . Severe outcome of juvenile idiopathic arthritis (JIA) associated with familial Mediterranean fever (FMF).Clin Exp Rheumatol2004; 22: S75 – S78 .
15.
15 Atagunduz P Ergun T Direskeneli H . MEFV mutations are increased in behcet's disease (BD) and are associated with vascular involvement.Clin Exp Rheumatol2003; 21: S35 – S37 .
16.
16 Shinar Y Livneh A Villa Y . Common mutations in the familial Mediterranean fever gene associate with rapid progression to disability in non-ashkenazi jewish multiple sclerosis patients.Genes Immun2003; 4: 197 – 203 .
17.
17 Ayvazyan A . Systemic lupus erythematosus, polyarteritis nodosa and familial Mediterranean fever in armenia.Ann Rheum Dis2006; 65: 255 .
18.
18 Bakir F Saaed B . Systemic lupus erythematosus and periodic peritonitis (FMF).Br J Rheumatol1989; 28: 81 – 82 .
19.
19 Langevitz P Livneh A Zemer D Dolitzky M Pras M . Systemic lupus erythematosus in patients with familial Mediterranean fever.Lupus1995; 4: 11 .
20.
20 Pauzner R Dulitzki M Langevitz P Livneh A Kenett R Many A . Low molecular weight heparin and warfarin in the treatment of patients with antiphospholipid syndrome during pregnancy.Thromb Haemost2001; 86: 1379 – 1384 .
21.
21 Lim KL Abdul-Wahab R Lowe J Powell RJ . Muscle biopsy abnormalities in systemic lupus erythematosus: correlation with clinical and laboratory parameters.Ann Rheum Dis1994; 53: 178 – 182 .
22.
22 Livneh A Langevitz P Zemer D . The changing face of familial Mediterranean fever.Semin Arthritis Rheum1996; 26: 612 – 627 .
23.
23 Zandman-Goddard G Blank M Langevitz P . Anti-serum amyloid component P antibodies in patients with systemic lupus erythematosus correlate with disease activity.Ann Rheum Dis2005; 64: 1698 – 1702 .
24.
24 Lidar M Kedem R Mor A Levartovsky D Langevitz P Livneh A . Arthritis as the sole episodic manifestation of familial Mediterranean fever.J Rheumatol2005; 32: 859 – 862 .
25.
25 Cronin ME . Musculoskeletal manifestations of systemic lupus erythematosus.Rheum Dis Clin North Am1988; 14: 99 – 116 .
26.
26 Cervera R Khamashta MA Font J . Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The european working party on systemic lupus erythematosus.Medicine (Baltimore)1993; 72: 113 – 124 .
27.
27 Ben-Chetrit E . Familial Mediterranean fever (FMF) and renal AA amyloidosis – phenotype-genotype correlation, treatment and prognosis.J Nephrol2003; 16: 431 – 434 .
28.
28 Dixon AJ Wall GC . Probable colchicine-induced neutropenia not related to intentional overdose.Ann Pharmacother2001; 35: 192 – 195 .
29.
29 Turkmen MSA Kavukcu S . Familial Mediterranean fever with atypical onset.Clin Exp Rheumatol2002; 20: 100 .
30.
30 Lachmann HJ Sengul B Yavuzsen TU . Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations.Rheumatology (Oxford)2006; 45: 746 – 750 .
31.
31 Emsley J White HE O'Hara BP . Structure of pentameric human serum amyloid P component.Nature1994; 367: 338 – 345 .
32.
32 Gillmore JD Hutchinson WL Herbert J . Autoimmunity and glomerulonephritis in mice with targeted deletion of the serum amyloid P component gene: SAP deficiency or strain combination?Immunology2004; 112: 255 – 264 .
33.
33 Lidar MDA Kedem R Yosepovich A Langevitz P Livneh A . Appendectomy in familial Mediterranean fever: clinical, genetic and pathological findings.Clin Exp Rheum2007; 2008, accepted.
34.
34 Ozen S Bakkaloglu A . C reactive protein: protecting from lupus in familial Mediterranean fever.Ann Rheum Dis2005; 64: 786 – 787 .
35.
35 Lachmann HJ Sengul B Yavuzsen TU . Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations.Rheumatology (Oxford)2006; 45: 746 – 750 .
36.
36 Suh CH Chun HY Ye YM Park HS . Unresponsiveness of C-reactive protein in the non-infectious inflammation of systemic lupus erythematosus is associated with interleukin 6.Clin Immunol2006; 119: 291 – 296 .
37.
37 Spronk PE Limburg PC Kallenberg CG . Serological markers of disease activity in systemic lupus erythematosus.Lupus1995; 4: 86 – 94 .
38.
38 Bijl M Bootsma H Van Der Geld Y Limburg PC Kallenberg CG Van Rijswijk MH . Serum amyloid P component levels are not decreased in patients with systemic lupus erythematosus and do not rise during an acute phase reaction.Ann Rheum Dis2004; 63: 831 – 835 .
39.
39 Abu-Shakra M Lorber M Neumann L Buskila D . Features of SLE in various ethnic groups in israel.Clin Exp Rheumatol1997; 15: 181 – 184 .
40.
40 Wysenbeek AJ Leibovici L Weinberger A Guedj D . Expression of systemic lupus erythematosus in various ethnic jewish israeli groups.Ann Rheum Dis1993; 52: 268 – 271 .
41.
41 Ben-Chetrit E Levy M . Colchicine: 1998 update.Semin Arthritis Rheum1998; 28: 48 – 59 .
