Hi - I am new to this forum.
I have had MGUS for 2 years and just recently was diagnosed with Sjogrens syndrome. I am having some trouble getting some information about the correlation of the two. Is it common? What are the percentage of people who have both where the MGUS turns into multiple myeloma?
Forums
8 posts
• Page 1 of 1
-
Alison214 - Name: Alison Holsman
- Who do you know with myeloma?: I have MGUS
- When were you/they diagnosed?: 2015
- Age at diagnosis: 49
Re: Sjogren's syndrome and MGUS
Hi Allison,
I am new to this forum as well. I have both Sjogren's and MGUS; however, mine appeared in the opposite order of yours. First the Sjogren's, then the MGUS ... much later. Yes, unfortunately there is a link between MGUS and Sjogren's. More information can be found in the article I've referenced below.
A couple of things I have learned. Take vitamin D; I read somewhere that those with Sjogren's who have low levels of vitamin D are more likely to develop NHL. Also, avoid live virus vaccinations; for whatever reason, they stimulate the immune system, which is the opposite of what we need. This is also the case if you are considering IVIG. In my case, I also have indolent non-Hodgkin's lymphoma in remission, another blood cancer Sjogren's patients are more likely to get.
Good luck.
Reference:
Shimanovsky, A, et al, "Autoimmune manifestations in patients with multiple myeloma and monoclonal gammopathy of undetermined significance," BBA Clinical, Dec 2016 (link).
Abstract:
Background - Multiple myeloma and its precursor, monoclonal gammopathy of undetermined significance (MGUS), have been linked with several autoimmune conditions in the medical literature. Yet, significance of these associations is not well understood.
Methods - Herein, we provide a comprehensive literature review on autoimmune disorders identified in patients with multiple myeloma and MGUS. Most relevant papers were identified via searching the PubMed/Medline and EMBASE databases for articles published from inception until May 1, 2016.
Findings - Scientific literature on autoimmune conditions in patients with multiple myeloma and MGUS consists of several case series and a multitude of case reports. Our analysis suggests an increased prevalence of autoimmune conditions in patients with multiple myeloma and monoclonal gammopathy of undetermined significance (MGUS), including various autoimmune hematologic and rheumatologic conditions among other entities. Conversely, persons with various autoimmune conditions tend to have a higher prevalence of MGUS and multiple myeloma than the general population.
Conclusions - Future research is required to explore further the link between MGUS / multiple myeloma and autoimmune disorders. Inflammation in the setting of autoimmunity may serve as a trigger for MGUS and multiple myeloma. In addition, a common genetic susceptibility for developing both an autoimmune disease and multiple myeloma / MGUS might also exist. Autoimmune hematologic and rheumatologic diseases may pose important clinical problems for the multiple myeloma patients. Therefore, a catalogue of these problems is important so that physicians are able to consider, identify and address them promptly.
I am new to this forum as well. I have both Sjogren's and MGUS; however, mine appeared in the opposite order of yours. First the Sjogren's, then the MGUS ... much later. Yes, unfortunately there is a link between MGUS and Sjogren's. More information can be found in the article I've referenced below.
A couple of things I have learned. Take vitamin D; I read somewhere that those with Sjogren's who have low levels of vitamin D are more likely to develop NHL. Also, avoid live virus vaccinations; for whatever reason, they stimulate the immune system, which is the opposite of what we need. This is also the case if you are considering IVIG. In my case, I also have indolent non-Hodgkin's lymphoma in remission, another blood cancer Sjogren's patients are more likely to get.
Good luck.
Reference:
Shimanovsky, A, et al, "Autoimmune manifestations in patients with multiple myeloma and monoclonal gammopathy of undetermined significance," BBA Clinical, Dec 2016 (link).
Abstract:
Background - Multiple myeloma and its precursor, monoclonal gammopathy of undetermined significance (MGUS), have been linked with several autoimmune conditions in the medical literature. Yet, significance of these associations is not well understood.
Methods - Herein, we provide a comprehensive literature review on autoimmune disorders identified in patients with multiple myeloma and MGUS. Most relevant papers were identified via searching the PubMed/Medline and EMBASE databases for articles published from inception until May 1, 2016.
Findings - Scientific literature on autoimmune conditions in patients with multiple myeloma and MGUS consists of several case series and a multitude of case reports. Our analysis suggests an increased prevalence of autoimmune conditions in patients with multiple myeloma and monoclonal gammopathy of undetermined significance (MGUS), including various autoimmune hematologic and rheumatologic conditions among other entities. Conversely, persons with various autoimmune conditions tend to have a higher prevalence of MGUS and multiple myeloma than the general population.
Conclusions - Future research is required to explore further the link between MGUS / multiple myeloma and autoimmune disorders. Inflammation in the setting of autoimmunity may serve as a trigger for MGUS and multiple myeloma. In addition, a common genetic susceptibility for developing both an autoimmune disease and multiple myeloma / MGUS might also exist. Autoimmune hematologic and rheumatologic diseases may pose important clinical problems for the multiple myeloma patients. Therefore, a catalogue of these problems is important so that physicians are able to consider, identify and address them promptly.
-
nellellen8052 - Name: Ellen
- Who do you know with myeloma?: Those on this forum
- When were you/they diagnosed?: MGUS 2016
- Age at diagnosis: 63
Re: Sjogren's syndrome and MGUS
Hi Alison,
It's not too hard to find articles like the one nellellen posted if you do an Internet search on keyword combinations like "myeloma Sjogren's".
There are also quite a few folks on this forum that suffer from Sjogren's, as you can see from threads like this one:
"Sjogren's syndrome and smoldering myeloma," started April 14, 2015
This study (which is referenced in nellellen's post above) suggests that up to two thirds of Sjogren's patients can develop a monoclonal gammopathy of some sort:
Moutsopoulos, ,HM, et al, "High incidence of free monoclonal lambda light chains in the sera of patients with Sjogren's syndrome," Journal of Immunology, June 1, 1983 (abstract)
Abstract:
Sjogren's syndrome presents a wide spectrum of disease manifestations ranging from benign to malignant lymphoproliferation. Sera from 21 patients with primary Sjogren's syndrome, 63 patients with other autoimmune rheumatic diseases, and 140 normal controls were studied by using high-resolution gel electrophoresis combined with immunofixation and specific absorption studies. Two-thirds of the sera from Sjogren's syndrome patients contained free monoclonal lambda light chains. In addition, one patient with Sjogren's syndrome and pseudolymphoma had a circulating monoclonal IgM-lambda immunoglobulin. In contrast, only 16% of the patients with other autoimmune diseases and 5% of normals had monoclonal bands; none of the other patients studied exhibited monoclonal-free lambda light chains in their serum. Our findings suggest that the monoclonal process in Sjogren's syndrome starts early in the disease process in a subset of B cells.
It's not too hard to find articles like the one nellellen posted if you do an Internet search on keyword combinations like "myeloma Sjogren's".
There are also quite a few folks on this forum that suffer from Sjogren's, as you can see from threads like this one:
"Sjogren's syndrome and smoldering myeloma," started April 14, 2015
This study (which is referenced in nellellen's post above) suggests that up to two thirds of Sjogren's patients can develop a monoclonal gammopathy of some sort:
Moutsopoulos, ,HM, et al, "High incidence of free monoclonal lambda light chains in the sera of patients with Sjogren's syndrome," Journal of Immunology, June 1, 1983 (abstract)
Abstract:
Sjogren's syndrome presents a wide spectrum of disease manifestations ranging from benign to malignant lymphoproliferation. Sera from 21 patients with primary Sjogren's syndrome, 63 patients with other autoimmune rheumatic diseases, and 140 normal controls were studied by using high-resolution gel electrophoresis combined with immunofixation and specific absorption studies. Two-thirds of the sera from Sjogren's syndrome patients contained free monoclonal lambda light chains. In addition, one patient with Sjogren's syndrome and pseudolymphoma had a circulating monoclonal IgM-lambda immunoglobulin. In contrast, only 16% of the patients with other autoimmune diseases and 5% of normals had monoclonal bands; none of the other patients studied exhibited monoclonal-free lambda light chains in their serum. Our findings suggest that the monoclonal process in Sjogren's syndrome starts early in the disease process in a subset of B cells.
-
Multibilly - Name: Multibilly
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Smoldering, Nov, 2012
Re: Sjogren's syndrome and MGUS
Thank you to both of you for the information. It gives me a place to start.
-
Alison214 - Name: Alison Holsman
- Who do you know with myeloma?: I have MGUS
- When were you/they diagnosed?: 2015
- Age at diagnosis: 49
Re: Sjogren's syndrome and MGUS
Hey Multibilly,
Thanks for your comments about Sjogren's syndromes and monoclonal gammopathies such as MGUS and multiple myeloma.
I think it's worth noting the year the paper was published that you cited. It's 1983. I mention it because, as far as I know, free light chain measurement at that point in time was not particularly advanced. So, in their abstract, the authors say they found "free monoclonal lambda light chains" in two thirds of the Sjogren's patients. They do not mention if the patients had serum free light chain levels that were above normal, and they admit to finding only one Sjogren patient (out of 21) that had a detectable M-spike.
I bring this up because I really wonder whether the presence of "monoclonal" free light chains in these patients is clinically significant beyond being a reflection of the patient's Sjogren's. More specifically, I am suspicious whether the observed free light chains are really due to an underlying plasma cell disorder.
I am fairly certain there are physiological conditions that can lead to low-levels of seemingly monoclonal free light chains and immunoglobulins being present in the blood, without an underlying plasma cell disorder being present. Some autoimmune disorders, perhaps including Sjogren's, may generate a narrowly differentiated immune response that manifests as seemingly monoclonal free and/or heavy chains, without their being any clonal plasma cells in the bone marrow.
In other words, having a very low level of monoclonal protein in the blood does not necessarily mean that someone has a plasma cell disorder.
I can't point to any published papers that reflect or describe in more detail what I just wrote. But I think it fits with what we often see here in the forum among people posting that they have autoimmune disorders that led them to also uncover low levels of monoclonal protein in their blood.
Also, I want to make it clear that I do not doubt the evidence indicating that people with autoimmune disorders are more likely to develop either smoldering or symptomatic multiple myeloma.
Thanks for your comments about Sjogren's syndromes and monoclonal gammopathies such as MGUS and multiple myeloma.
I think it's worth noting the year the paper was published that you cited. It's 1983. I mention it because, as far as I know, free light chain measurement at that point in time was not particularly advanced. So, in their abstract, the authors say they found "free monoclonal lambda light chains" in two thirds of the Sjogren's patients. They do not mention if the patients had serum free light chain levels that were above normal, and they admit to finding only one Sjogren patient (out of 21) that had a detectable M-spike.
I bring this up because I really wonder whether the presence of "monoclonal" free light chains in these patients is clinically significant beyond being a reflection of the patient's Sjogren's. More specifically, I am suspicious whether the observed free light chains are really due to an underlying plasma cell disorder.
I am fairly certain there are physiological conditions that can lead to low-levels of seemingly monoclonal free light chains and immunoglobulins being present in the blood, without an underlying plasma cell disorder being present. Some autoimmune disorders, perhaps including Sjogren's, may generate a narrowly differentiated immune response that manifests as seemingly monoclonal free and/or heavy chains, without their being any clonal plasma cells in the bone marrow.
In other words, having a very low level of monoclonal protein in the blood does not necessarily mean that someone has a plasma cell disorder.
I can't point to any published papers that reflect or describe in more detail what I just wrote. But I think it fits with what we often see here in the forum among people posting that they have autoimmune disorders that led them to also uncover low levels of monoclonal protein in their blood.
Also, I want to make it clear that I do not doubt the evidence indicating that people with autoimmune disorders are more likely to develop either smoldering or symptomatic multiple myeloma.
-
JimNY
Re: Sjogren's syndrome and MGUS
Thanks for pointing this out Jim. All valid points and I agree with your concerns. Here is a more recent study that touches on the subject.
https://www.ncbi.nlm.nih.gov/pubmed/22297146
The percentage of Sjogrens's patients in this study with a monoclonal gammopathy was 22%. In those patients with HCV (hepatitis C) associated Sojgren's, the percentage with a monoclonal gammopathy jumps to 52%. Of the 22% of patients with a monoclonal gammopathy, 10/48 (about 21%) went on to develop a neoplasia in 10 years.
METHODS:
Serum immunoelectrophoresis (IE) was performed to 408 consecutive patients who were evaluated by our unit between 1992 and 2011: 221 patients who fulfilled the 2002 American-European criteria for primary SS, 122 primary SS patients who fulfilled exclusively the 1993 European criteria and 65 patients with SS-associated hepatitis C virus infection. IE was performed at diagnosis and every year during the follow-up.
RESULTS:
Of the 221 patients with primary SS, 48 (22%) had monoclonal gammopathy. In the control groups, the prevalence was 16% in patients with SS who fulfilled the 1993 criteria (p > 0.05) and 52% in SS-HCV patients (p < 0.001). Monoclonal bands were characterized in 47/48 patients with primary SS: IgG (n = 21), IgM (n = 16), IgA (n = 5) and free light chains (n = 5); the light chain was κ in 28 patients and λ in 19 (κ:λ ratio 1.5). Primary SS patients with monoclonal gammopathy had a higher prevalence of parotidomegaly (38% vs 20%, p = 0.021), vasculitis (21% vs 6%, p = 0.003), neurological involvement (42% vs 23%, p = 0.016), higher mean values of circulating gammaglobulins (23.4 vs 20.6%, p = 0.026), ESR (56.6 vs 37.6 multiple myeloma/h, p = 0.003), a higher prevalence of RF (69% vs 50%, p = 0.022), low C3 levels (24% vs 11%, p = 0.028), low C4 levels (24% vs 7%, p = 0.003), low CH50 activity (28% vs 11%, p = 0.008) and cryoglobulins (23% vs 8%, p = 0.012) compared with those without monoclonal gammopathy. Of the 48 patients with primary SS and monoclonal gammopathy, 8 developed hematologic neoplasia after a mean follow-up of 10 years, a higher prevalence than observed in patients without monoclonal gammopathy (17% vs 5%, p = 0.009). Survival rates according to the presence or absence of monoclonal gammopathy were 83% and 97%, respectively (log rank 0.004).
CONCLUSION:
Monoclonal gammopathy was detected in 22% of patients with primary SS fulfilling the 2002 criteria, with mIgGκ being the most frequent type of band detected. In HCV-associated SS patients, the prevalence was higher (52%) with IgMκ being the most prevalent band detected. Monoclonal gammopathy was associated with a higher prevalence of parotid enlargement, extraglandular features, hypergammaglobulinemia, cryoglobulinemia and related markers (rheumatoid factor, hypocomplementemia), and with a poor prognosis (development of neoplasia and death).
https://www.ncbi.nlm.nih.gov/pubmed/22297146
The percentage of Sjogrens's patients in this study with a monoclonal gammopathy was 22%. In those patients with HCV (hepatitis C) associated Sojgren's, the percentage with a monoclonal gammopathy jumps to 52%. Of the 22% of patients with a monoclonal gammopathy, 10/48 (about 21%) went on to develop a neoplasia in 10 years.
METHODS:
Serum immunoelectrophoresis (IE) was performed to 408 consecutive patients who were evaluated by our unit between 1992 and 2011: 221 patients who fulfilled the 2002 American-European criteria for primary SS, 122 primary SS patients who fulfilled exclusively the 1993 European criteria and 65 patients with SS-associated hepatitis C virus infection. IE was performed at diagnosis and every year during the follow-up.
RESULTS:
Of the 221 patients with primary SS, 48 (22%) had monoclonal gammopathy. In the control groups, the prevalence was 16% in patients with SS who fulfilled the 1993 criteria (p > 0.05) and 52% in SS-HCV patients (p < 0.001). Monoclonal bands were characterized in 47/48 patients with primary SS: IgG (n = 21), IgM (n = 16), IgA (n = 5) and free light chains (n = 5); the light chain was κ in 28 patients and λ in 19 (κ:λ ratio 1.5). Primary SS patients with monoclonal gammopathy had a higher prevalence of parotidomegaly (38% vs 20%, p = 0.021), vasculitis (21% vs 6%, p = 0.003), neurological involvement (42% vs 23%, p = 0.016), higher mean values of circulating gammaglobulins (23.4 vs 20.6%, p = 0.026), ESR (56.6 vs 37.6 multiple myeloma/h, p = 0.003), a higher prevalence of RF (69% vs 50%, p = 0.022), low C3 levels (24% vs 11%, p = 0.028), low C4 levels (24% vs 7%, p = 0.003), low CH50 activity (28% vs 11%, p = 0.008) and cryoglobulins (23% vs 8%, p = 0.012) compared with those without monoclonal gammopathy. Of the 48 patients with primary SS and monoclonal gammopathy, 8 developed hematologic neoplasia after a mean follow-up of 10 years, a higher prevalence than observed in patients without monoclonal gammopathy (17% vs 5%, p = 0.009). Survival rates according to the presence or absence of monoclonal gammopathy were 83% and 97%, respectively (log rank 0.004).
CONCLUSION:
Monoclonal gammopathy was detected in 22% of patients with primary SS fulfilling the 2002 criteria, with mIgGκ being the most frequent type of band detected. In HCV-associated SS patients, the prevalence was higher (52%) with IgMκ being the most prevalent band detected. Monoclonal gammopathy was associated with a higher prevalence of parotid enlargement, extraglandular features, hypergammaglobulinemia, cryoglobulinemia and related markers (rheumatoid factor, hypocomplementemia), and with a poor prognosis (development of neoplasia and death).
-
Multibilly - Name: Multibilly
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Smoldering, Nov, 2012
Re: Sjogren's syndrome and MGUS
I was diagnosed with Sjogren's several years back (plus other autoimmune issues) My worst problems are joint pain, the dry skin, mouth and eyes. Eyes, to the point being soooo dry, that my corneas were very messed up with lots of corneal abrasions that led to eye surgeries to poke many, many tiny holes and let it heal over. Not fun at all! Didn't feel it being done, but after the numbing wears off and the days of healing after – whoa, painkillers. lol
But, it worked and I now can wear contacts, which are mainly to now protect my corneas from the tiny little punctal plugs I have in my tear-ducts, because my corneas are still so sensitive, when the contacts are out, the minuscule tips of the plugs that stick out, can tear open my corneas again. Even with the plug I still need to use artificial tears a few times a day and especially upon waking up, because I have to keep my contacts in 24/7/365, which makes my eyes dryer in the mornings. I keep bottles of artificial tears in various places to have at hand real fast because just opening my eyes in the morning can move the contacts and, bam, rip goes a cornea! :/
But anyway, the MGUS diagnosis came only recently, so much after the Sjogren's diagnosis. But, my myeloma specialist did admit to me that having Sjogrens does indeed increase my chance of my MGUS turning into multiple myeloma some day. He also said the fact that I'm IgA kappa increases it as well.
But, it worked and I now can wear contacts, which are mainly to now protect my corneas from the tiny little punctal plugs I have in my tear-ducts, because my corneas are still so sensitive, when the contacts are out, the minuscule tips of the plugs that stick out, can tear open my corneas again. Even with the plug I still need to use artificial tears a few times a day and especially upon waking up, because I have to keep my contacts in 24/7/365, which makes my eyes dryer in the mornings. I keep bottles of artificial tears in various places to have at hand real fast because just opening my eyes in the morning can move the contacts and, bam, rip goes a cornea! :/
But anyway, the MGUS diagnosis came only recently, so much after the Sjogren's diagnosis. But, my myeloma specialist did admit to me that having Sjogrens does indeed increase my chance of my MGUS turning into multiple myeloma some day. He also said the fact that I'm IgA kappa increases it as well.
-
CathyCav - Name: Cathy
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: DXd w/MGUS Jan 2017
- Age at diagnosis: 50
Re: Sjogren's syndrome and MGUS
This is a brand new article on the subject of Sjogren's and cancer risk.
Reference:
Brito-Zerón, P, et al, "Characterization and risk estimate of cancer in patients with primary Sjögren syndrome," Journal of Hematology & Oncology, April 2017 (full text of article)
Abstract:
Background: The purpose of this study is to characterize the risk of cancer in a large cohort of patients with primary Sjögren syndrome (SjS).
Methods: We had analyzed the development of cancer in 1300 consecutive patients fulfilling the 2002 SjS classification criteria. The baseline clinical and immunological characteristics and systemic activity (ESSDAI scores) were assessed at diagnosis as predictors of cancer using Cox proportional hazards regression analysis adjusted for age at diagnosis and gender. The sex-and age-specific standardized incidence ratios (SIR) of cancer were estimated from 2012 Spanish mortality data.
Results: After a mean follow-up of 91 months, 127 (9.8%) patients developed 133 cancers. The most frequent type of cancer was B-cell lymphoma (including 27 MALT and 19 non-MALT B-cell lymphomas). Systemic activity at diagnosis of primary SjS correlated with the risk of hematological neoplasia and cryoglobulins with a high risk of either B-cell or non-B-cell lymphoma subtypes. Patients with cytopenias had a high risk of non-MALT B-cell and non-B-cell cancer, while those with low C3 levels had a high risk of MALT lymphomas and those with monoclonal gammopathy and low C4 levels had a high risk of non-MALT lymphomas. The estimated SIR for solid cancer was 1.13 and 11.02 for hematological cancer. SIRs for specific cancers were 36.17 for multiple myeloma and immunoproliferative diseases, 19.41 for Hodgkin lymphoma, 6.04 for other non-Hodgkin lymphomas, 5.17 for thyroid cancer, 4.81 for cancers of the lip and oral cavity, and 2.53 for stomach cancer.
Conclusions: One third of cancers developed by patients with primary SjS are B-cell lymphomas. The prognostic factors identified at SjS diagnosis differed according to the subtype of B-cell lymphoma developed. Primary SjS is also associated with the development of some non-hematological cancers (thyroid, oral cavity, and stomach).
Reference:
Brito-Zerón, P, et al, "Characterization and risk estimate of cancer in patients with primary Sjögren syndrome," Journal of Hematology & Oncology, April 2017 (full text of article)
Abstract:
Background: The purpose of this study is to characterize the risk of cancer in a large cohort of patients with primary Sjögren syndrome (SjS).
Methods: We had analyzed the development of cancer in 1300 consecutive patients fulfilling the 2002 SjS classification criteria. The baseline clinical and immunological characteristics and systemic activity (ESSDAI scores) were assessed at diagnosis as predictors of cancer using Cox proportional hazards regression analysis adjusted for age at diagnosis and gender. The sex-and age-specific standardized incidence ratios (SIR) of cancer were estimated from 2012 Spanish mortality data.
Results: After a mean follow-up of 91 months, 127 (9.8%) patients developed 133 cancers. The most frequent type of cancer was B-cell lymphoma (including 27 MALT and 19 non-MALT B-cell lymphomas). Systemic activity at diagnosis of primary SjS correlated with the risk of hematological neoplasia and cryoglobulins with a high risk of either B-cell or non-B-cell lymphoma subtypes. Patients with cytopenias had a high risk of non-MALT B-cell and non-B-cell cancer, while those with low C3 levels had a high risk of MALT lymphomas and those with monoclonal gammopathy and low C4 levels had a high risk of non-MALT lymphomas. The estimated SIR for solid cancer was 1.13 and 11.02 for hematological cancer. SIRs for specific cancers were 36.17 for multiple myeloma and immunoproliferative diseases, 19.41 for Hodgkin lymphoma, 6.04 for other non-Hodgkin lymphomas, 5.17 for thyroid cancer, 4.81 for cancers of the lip and oral cavity, and 2.53 for stomach cancer.
Conclusions: One third of cancers developed by patients with primary SjS are B-cell lymphomas. The prognostic factors identified at SjS diagnosis differed according to the subtype of B-cell lymphoma developed. Primary SjS is also associated with the development of some non-hematological cancers (thyroid, oral cavity, and stomach).
-
Multibilly - Name: Multibilly
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Smoldering, Nov, 2012
8 posts
• Page 1 of 1