Immune-related and inflammatory conditions and risk of lymphoplasmacytic lymphoma or Waldenstrom macroglobulinemia.
BACKGROUND
Chronic immune stimulation appears to be associated with lymphoplasmacytic lymphoma (LPL)-Waldenström macroglobulinemia (WM); however, available information is sparse. We conducted, to our knowledge, the most comprehensive study to date to evaluate associations between a personal or family history of many immune-related and/or inflammatory disorders and the subsequent risk of LPL-WM.


METHODS
We used Swedish population-based registries to identify 2470 case patients with LPL-WM, 9698 matched control subjects, and almost 30 000 first-degree relatives of either case patients or control subjects. We evaluated a wide range of autoimmune, infectious, allergic, and inflammatory conditions. We calculated odds ratios (ORs) and 95% confidence intervals (CIs) for each condition by use of logistic regression.


RESULTS
An increased risk of LPL-WM was associated with a personal history of the following autoimmune diseases: systemic sclerosis (OR = 4.7, 95% CI = 1.4 to 15.3), Sjögren syndrome (OR = 12.1, 95% CI = 3.3 to 45.0), autoimmune hemolytic anemia (OR = 24.2, 95% CI = 5.4 to 108.2), polymyalgia rheumatica (OR = 2.9, 95% CI = 1.6 to 5.2), and giant cell arteritis (OR = 8.3, 95% CI = 2.1 to 33.1). An increased risk of LPL-WM was associated with a personal history of the following infectious diseases: pneumonia (OR = 1.4, 95% CI = 1.1 to 1.7), septicemia (OR = 2.4, 95% CI = 1.2 to 4.3), pyelonephritis (OR = 1.7, 95% CI = 1.1 to 2.5), sinusitis (OR = 2.7, 95% CI = 1.4 to 4.9), herpes zoster (OR = 3.4, 95% CI = 2.0 to 5.6), and influenza (OR = 2.9, 95% CI = 1.7 to 5.0). An increased risk of LPL-WM was associated with a family history of the following autoimmune or infectious diseases: Sjögren syndrome (OR = 5.0, 95% CI = 2.1 to 12.0), autoimmune hemolytic anemia (OR = 3.8, 95% CI = 1.1 to 13.2), Guillain-Barré syndrome (OR = 4.1, 95% CI = 1.8 to 9.4), cytomegalovirus (OR = 2.7, 95% CI = 1.4 to 5.3), gingivitis and periodontitis (OR = 1.9, 95% CI = 1.3 to 2.7), and chronic prostatitis (OR = 4.3, 95% CI = 1.7 to 11.1).


CONCLUSIONS
Personal history of certain immune-related and/or infectious conditions was strongly associated with increased risk of LPL-WM. The association of both personal and family history of Sjögren syndrome and autoimmune hemolytic anemia with risk of LPL-WM indicates the potential for shared susceptibility for these conditions.
Kristinsson
Sigurdur Y
SY
Department of Medicine, Karolinska University Hospital Solna, SE-171 76 Stockholm, Sweden. sigurdur.kristinsson@karolinska.se
Koshiol
Jill
J
Björkholm
Magnus
M
Goldin
Lynn R
LR
McMaster
Mary L
ML
Turesson
Ingemar
I
Landgren
Ola
O
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications; D001327:Autoimmune Diseases / Q000150:complications*; D016022:Case-Control Studies; D002908:Chronic Disease; D016001:Confidence Intervals; D003586:Cytomegalovirus Infections / Q000150:complications; D004198:Disease Susceptibility; D005260:Female; D005891:Gingivitis / Q000150:complications; D020275:Guillain-Barre Syndrome / Q000150:complications; D006562:Herpes Zoster / Q000150:complications; D006801:Humans; D006967:Hypersensitivity / Q000150:complications; D007239:Infections / Q000150:complications*; D007251:Influenza, Human / Q000150:complications; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D016017:Odds Ratio; D010518:Periodontitis / Q000150:complications; D011014:Pneumonia / Q000150:complications; D011472:Prostatitis / Q000150:complications; D011704:Pyelonephritis / Q000150:complications; D012042:Registries; D018570:Risk Assessment; D012307:Risk Factors; D018805:Sepsis / Q000150:complications; D012852:Sinusitis / Q000150:complications; D012859:Sjogren's Syndrome / Q000150:complications; D013548:Sweden / Q000453:epidemiology; D008258:Waldenstrom Macroglobulinemia / Q000453:epidemiology* / Q000209:etiology* / Q000276:immunology