Free light chains of immunoglobulins in patients with systemic sclerosis: Correlations with lung involvement and inflammatory milieu

Silvia Bosello, Umberto Basile, Enrico De Lorenzis, Francesca Gulli, Giovanni Canestrari, Cecilia Napodano, Federico Parisi, Krizia Pocino, Clara Di Mario, Barbara Tolusso, Gianfranco Ferraccioli, Elisa Gremese

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Aim: Humoral immunity and B cells are thought to play an important role in the pathophysiology of the systemic sclerosis (SSc). The production of free light chains (FLC) of immunoglobulins is abnormally high in several pathological autoimmune conditions and reflects B cell activation. Furthermore, FLCs demonstrated different biological activities including their capability to modulate the immune system, proteolytic activity and complement cascade activation. The aims of this study are to determine the FLC levels in patients with SSc compared with healthy controls (HC) and to study their possible association with organ involvement and disease characteristics. Methods: Sixty-five patients with SSc and 20 HC were studied. Clinical and immunological inflammatory characteristics were assessed for all the patients with SSc. κ -FLC and λ -FLC, interleukin 6 (IL-6) and B cell activating factor levels were measured. Results: The mean serum κ -FLC levels and FLC ratio were significantly higher in patients with SSc compared with HC, while the serum λ -FLC levels were comparable. The levels of FLC were comparable in patients with diffuse skin disease and limited skin involvement, while κ -FLC levels were increased in patients with restrictive lung (forced vital capacity (FVC) <80%) disease (26.4±7.4 mg/L) when compared with patients with FVC ≥80% (19.6±7.3 mg/L, P=0.009). In patients with SSc, the levels of serum κ -FLC level directly correlated with the IL-6 levels (R=0.3, P=0.001) and disease activity (R=0.4, P=0.003). Conclusions: FLC levels are elevated in SSc and high levels are associated with lung involvement and with a higher degree of inflammation, supporting a possible role of B cell activation in the pathophysiology of the disease.

Original languageEnglish
JournalJournal of Clinical Pathology
DOIs
Publication statusAccepted/In press - Jan 31 2018

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Immunoglobulin Light Chains
Systemic Scleroderma
Light
Lung
B-Lymphocytes
Vital Capacity
Interleukin-6
Serum
B-Cell Activating Factor
Complement Activation
Humoral Immunity
Skin Diseases
Immune System
Inflammation

Keywords

  • autoantibody
  • autoimmune laboratory investigations
  • autoimmunity

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Free light chains of immunoglobulins in patients with systemic sclerosis : Correlations with lung involvement and inflammatory milieu. / Bosello, Silvia; Basile, Umberto; De Lorenzis, Enrico; Gulli, Francesca; Canestrari, Giovanni; Napodano, Cecilia; Parisi, Federico; Pocino, Krizia; Di Mario, Clara; Tolusso, Barbara; Ferraccioli, Gianfranco; Gremese, Elisa.

In: Journal of Clinical Pathology, 31.01.2018.

Research output: Contribution to journalArticle

Bosello, Silvia ; Basile, Umberto ; De Lorenzis, Enrico ; Gulli, Francesca ; Canestrari, Giovanni ; Napodano, Cecilia ; Parisi, Federico ; Pocino, Krizia ; Di Mario, Clara ; Tolusso, Barbara ; Ferraccioli, Gianfranco ; Gremese, Elisa. / Free light chains of immunoglobulins in patients with systemic sclerosis : Correlations with lung involvement and inflammatory milieu. In: Journal of Clinical Pathology. 2018.
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abstract = "Aim: Humoral immunity and B cells are thought to play an important role in the pathophysiology of the systemic sclerosis (SSc). The production of free light chains (FLC) of immunoglobulins is abnormally high in several pathological autoimmune conditions and reflects B cell activation. Furthermore, FLCs demonstrated different biological activities including their capability to modulate the immune system, proteolytic activity and complement cascade activation. The aims of this study are to determine the FLC levels in patients with SSc compared with healthy controls (HC) and to study their possible association with organ involvement and disease characteristics. Methods: Sixty-five patients with SSc and 20 HC were studied. Clinical and immunological inflammatory characteristics were assessed for all the patients with SSc. κ -FLC and λ -FLC, interleukin 6 (IL-6) and B cell activating factor levels were measured. Results: The mean serum κ -FLC levels and FLC ratio were significantly higher in patients with SSc compared with HC, while the serum λ -FLC levels were comparable. The levels of FLC were comparable in patients with diffuse skin disease and limited skin involvement, while κ -FLC levels were increased in patients with restrictive lung (forced vital capacity (FVC) <80{\%}) disease (26.4±7.4 mg/L) when compared with patients with FVC ≥80{\%} (19.6±7.3 mg/L, P=0.009). In patients with SSc, the levels of serum κ -FLC level directly correlated with the IL-6 levels (R=0.3, P=0.001) and disease activity (R=0.4, P=0.003). Conclusions: FLC levels are elevated in SSc and high levels are associated with lung involvement and with a higher degree of inflammation, supporting a possible role of B cell activation in the pathophysiology of the disease.",
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author = "Silvia Bosello and Umberto Basile and {De Lorenzis}, Enrico and Francesca Gulli and Giovanni Canestrari and Cecilia Napodano and Federico Parisi and Krizia Pocino and {Di Mario}, Clara and Barbara Tolusso and Gianfranco Ferraccioli and Elisa Gremese",
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T1 - Free light chains of immunoglobulins in patients with systemic sclerosis

T2 - Correlations with lung involvement and inflammatory milieu

AU - Bosello, Silvia

AU - Basile, Umberto

AU - De Lorenzis, Enrico

AU - Gulli, Francesca

AU - Canestrari, Giovanni

AU - Napodano, Cecilia

AU - Parisi, Federico

AU - Pocino, Krizia

AU - Di Mario, Clara

AU - Tolusso, Barbara

AU - Ferraccioli, Gianfranco

AU - Gremese, Elisa

PY - 2018/1/31

Y1 - 2018/1/31

N2 - Aim: Humoral immunity and B cells are thought to play an important role in the pathophysiology of the systemic sclerosis (SSc). The production of free light chains (FLC) of immunoglobulins is abnormally high in several pathological autoimmune conditions and reflects B cell activation. Furthermore, FLCs demonstrated different biological activities including their capability to modulate the immune system, proteolytic activity and complement cascade activation. The aims of this study are to determine the FLC levels in patients with SSc compared with healthy controls (HC) and to study their possible association with organ involvement and disease characteristics. Methods: Sixty-five patients with SSc and 20 HC were studied. Clinical and immunological inflammatory characteristics were assessed for all the patients with SSc. κ -FLC and λ -FLC, interleukin 6 (IL-6) and B cell activating factor levels were measured. Results: The mean serum κ -FLC levels and FLC ratio were significantly higher in patients with SSc compared with HC, while the serum λ -FLC levels were comparable. The levels of FLC were comparable in patients with diffuse skin disease and limited skin involvement, while κ -FLC levels were increased in patients with restrictive lung (forced vital capacity (FVC) <80%) disease (26.4±7.4 mg/L) when compared with patients with FVC ≥80% (19.6±7.3 mg/L, P=0.009). In patients with SSc, the levels of serum κ -FLC level directly correlated with the IL-6 levels (R=0.3, P=0.001) and disease activity (R=0.4, P=0.003). Conclusions: FLC levels are elevated in SSc and high levels are associated with lung involvement and with a higher degree of inflammation, supporting a possible role of B cell activation in the pathophysiology of the disease.

AB - Aim: Humoral immunity and B cells are thought to play an important role in the pathophysiology of the systemic sclerosis (SSc). The production of free light chains (FLC) of immunoglobulins is abnormally high in several pathological autoimmune conditions and reflects B cell activation. Furthermore, FLCs demonstrated different biological activities including their capability to modulate the immune system, proteolytic activity and complement cascade activation. The aims of this study are to determine the FLC levels in patients with SSc compared with healthy controls (HC) and to study their possible association with organ involvement and disease characteristics. Methods: Sixty-five patients with SSc and 20 HC were studied. Clinical and immunological inflammatory characteristics were assessed for all the patients with SSc. κ -FLC and λ -FLC, interleukin 6 (IL-6) and B cell activating factor levels were measured. Results: The mean serum κ -FLC levels and FLC ratio were significantly higher in patients with SSc compared with HC, while the serum λ -FLC levels were comparable. The levels of FLC were comparable in patients with diffuse skin disease and limited skin involvement, while κ -FLC levels were increased in patients with restrictive lung (forced vital capacity (FVC) <80%) disease (26.4±7.4 mg/L) when compared with patients with FVC ≥80% (19.6±7.3 mg/L, P=0.009). In patients with SSc, the levels of serum κ -FLC level directly correlated with the IL-6 levels (R=0.3, P=0.001) and disease activity (R=0.4, P=0.003). Conclusions: FLC levels are elevated in SSc and high levels are associated with lung involvement and with a higher degree of inflammation, supporting a possible role of B cell activation in the pathophysiology of the disease.

KW - autoantibody

KW - autoimmune laboratory investigations

KW - autoimmunity

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U2 - 10.1136/jclinpath-2017-204656

DO - 10.1136/jclinpath-2017-204656

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