Paraproteinemic maculopathy

Ahmad M. Mansour, J. Fernando Arevalo, Josep Badal, Ramana S. Moorthy, Gaurav K. Shah, Hernando Zegarra, Jose S Pulido, Abdulrazzak Charbaji, Luis Amselem, Alejandro Jose Lavaque, Antonio Casella, Baseer Ahmad, Joshua G. Paschall, Antonio Caimi, Giovanni Staurenghi

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Purpose: Paraproteinemia relates to monoclonal gammopathy-producing pathologic antibodies with serous macular detachment being an uncommon ocular manifestation. We ascertained the clinical course of maculopathy in paraproteinemia and investigated the effect of various therapeutic methods on the resolution of subretinal deposits.

Design: Multicenter, retrospective, observational case series.

Participants: The records of patients with paraproteinemia with optical coherence tomography (OCT) documentation of serous macular detachment were reviewed.

Methods: Data collection included coexisting morbidity, rheology data (immunoglobulin level, hematocrit, and blood viscosity), clinical examination results, and OCT findings.

Main Outcome Measures: Best-corrected visual acuity (BCVA), height and basal area of the serous macular detachment, and systemic versus local therapies.

Results: A total of 33 cases were collected: 10 new and 23 previously reported in the literature. Diabetes was present in 7 patients, systemic hypertension in 9 patients, and anemia in 18. Mean initial immunoglobulin level was 6497 mg/dl, and mean serum viscosity was 5.5 centipoise (cP). Mean logarithm of the minimum angle of resolution initial vs. final BCVA was 0.55 (Snellen equivalent, 20/71) vs. 0.45 (20/56) in the right eye and 0.38 (20/48) vs. 0.50 (20/63) in the left eye. After mean follow-up of 7 months (range, 0-51 months). Systemic therapies included plasmapheresis (18), chemotherapy (30), blood transfusions (2), transplantation of progenitor hematopoietic cells (2), and oral rituximab (10). Immunoglobulin levels normalized in 8 patients and were unchanged in 1 after plasmapheresis, chemotherapy, or both. Ocular therapy in 8 patients included vitrectomy (1), laser photocoagulation (4), intravitreal bevacizumab (5), intravitreal triamcinolone (2), intravitreal dexamethasone implant (1), intravitreal rituximab (1), and sub-Tenon corticosteroid (1). The maculopathy resolved partially or completely in 17 patients and worsened or remained unchanged in 14 patients over median follow-up of 7 months. Maculopathy was unilateral in 9 cases and occurred at a lower initial immunoglobulin level in diabetics. There was a positive correlation between area of the detachment and serum viscosity.

Conclusions: Paraproteinemicmaculopathy can be unilateral. Decreasing the blood immunoglobulin level is the primary goal of therapy for paraproteinemic maculopathy, and this can be achieved by a systemic route. Coexisting diabetes facilitates leakage of immunoglobulins at lower levels than in nondiabetics.

Original languageEnglish (US)
Pages (from-to)1925-1932
Number of pages8
JournalOphthalmology
Volume121
Issue number10
DOIs
StatePublished - Oct 1 2014

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Immunoglobulins
Paraproteinemias
Plasmapheresis
Optical Coherence Tomography
Viscosity
Visual Acuity
Eye Manifestations
Triamcinolone
Drug Therapy
Blood Viscosity
Rheology
Light Coagulation
Vitrectomy
Therapeutic Uses
Therapeutics
Hematopoietic Stem Cells
Serum
Hematocrit
Blood Transfusion
Documentation

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Mansour, A. M., Arevalo, J. F., Badal, J., Moorthy, R. S., Shah, G. K., Zegarra, H., ... Staurenghi, G. (2014). Paraproteinemic maculopathy. Ophthalmology, 121(10), 1925-1932. https://doi.org/10.1016/j.ophtha.2014.04.007

Paraproteinemic maculopathy. / Mansour, Ahmad M.; Arevalo, J. Fernando; Badal, Josep; Moorthy, Ramana S.; Shah, Gaurav K.; Zegarra, Hernando; Pulido, Jose S; Charbaji, Abdulrazzak; Amselem, Luis; Lavaque, Alejandro Jose; Casella, Antonio; Ahmad, Baseer; Paschall, Joshua G.; Caimi, Antonio; Staurenghi, Giovanni.

In: Ophthalmology, Vol. 121, No. 10, 01.10.2014, p. 1925-1932.

Research output: Contribution to journalArticle

Mansour, AM, Arevalo, JF, Badal, J, Moorthy, RS, Shah, GK, Zegarra, H, Pulido, JS, Charbaji, A, Amselem, L, Lavaque, AJ, Casella, A, Ahmad, B, Paschall, JG, Caimi, A & Staurenghi, G 2014, 'Paraproteinemic maculopathy', Ophthalmology, vol. 121, no. 10, pp. 1925-1932. https://doi.org/10.1016/j.ophtha.2014.04.007
Mansour AM, Arevalo JF, Badal J, Moorthy RS, Shah GK, Zegarra H et al. Paraproteinemic maculopathy. Ophthalmology. 2014 Oct 1;121(10):1925-1932. https://doi.org/10.1016/j.ophtha.2014.04.007
Mansour, Ahmad M. ; Arevalo, J. Fernando ; Badal, Josep ; Moorthy, Ramana S. ; Shah, Gaurav K. ; Zegarra, Hernando ; Pulido, Jose S ; Charbaji, Abdulrazzak ; Amselem, Luis ; Lavaque, Alejandro Jose ; Casella, Antonio ; Ahmad, Baseer ; Paschall, Joshua G. ; Caimi, Antonio ; Staurenghi, Giovanni. / Paraproteinemic maculopathy. In: Ophthalmology. 2014 ; Vol. 121, No. 10. pp. 1925-1932.
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abstract = "Purpose: Paraproteinemia relates to monoclonal gammopathy-producing pathologic antibodies with serous macular detachment being an uncommon ocular manifestation. We ascertained the clinical course of maculopathy in paraproteinemia and investigated the effect of various therapeutic methods on the resolution of subretinal deposits.Design: Multicenter, retrospective, observational case series.Participants: The records of patients with paraproteinemia with optical coherence tomography (OCT) documentation of serous macular detachment were reviewed.Methods: Data collection included coexisting morbidity, rheology data (immunoglobulin level, hematocrit, and blood viscosity), clinical examination results, and OCT findings.Main Outcome Measures: Best-corrected visual acuity (BCVA), height and basal area of the serous macular detachment, and systemic versus local therapies.Results: A total of 33 cases were collected: 10 new and 23 previously reported in the literature. Diabetes was present in 7 patients, systemic hypertension in 9 patients, and anemia in 18. Mean initial immunoglobulin level was 6497 mg/dl, and mean serum viscosity was 5.5 centipoise (cP). Mean logarithm of the minimum angle of resolution initial vs. final BCVA was 0.55 (Snellen equivalent, 20/71) vs. 0.45 (20/56) in the right eye and 0.38 (20/48) vs. 0.50 (20/63) in the left eye. After mean follow-up of 7 months (range, 0-51 months). Systemic therapies included plasmapheresis (18), chemotherapy (30), blood transfusions (2), transplantation of progenitor hematopoietic cells (2), and oral rituximab (10). Immunoglobulin levels normalized in 8 patients and were unchanged in 1 after plasmapheresis, chemotherapy, or both. Ocular therapy in 8 patients included vitrectomy (1), laser photocoagulation (4), intravitreal bevacizumab (5), intravitreal triamcinolone (2), intravitreal dexamethasone implant (1), intravitreal rituximab (1), and sub-Tenon corticosteroid (1). The maculopathy resolved partially or completely in 17 patients and worsened or remained unchanged in 14 patients over median follow-up of 7 months. Maculopathy was unilateral in 9 cases and occurred at a lower initial immunoglobulin level in diabetics. There was a positive correlation between area of the detachment and serum viscosity.Conclusions: Paraproteinemicmaculopathy can be unilateral. Decreasing the blood immunoglobulin level is the primary goal of therapy for paraproteinemic maculopathy, and this can be achieved by a systemic route. Coexisting diabetes facilitates leakage of immunoglobulins at lower levels than in nondiabetics.",
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T1 - Paraproteinemic maculopathy

AU - Mansour, Ahmad M.

AU - Arevalo, J. Fernando

AU - Badal, Josep

AU - Moorthy, Ramana S.

AU - Shah, Gaurav K.

AU - Zegarra, Hernando

AU - Pulido, Jose S

AU - Charbaji, Abdulrazzak

AU - Amselem, Luis

AU - Lavaque, Alejandro Jose

AU - Casella, Antonio

AU - Ahmad, Baseer

AU - Paschall, Joshua G.

AU - Caimi, Antonio

AU - Staurenghi, Giovanni

PY - 2014/10/1

Y1 - 2014/10/1

N2 - Purpose: Paraproteinemia relates to monoclonal gammopathy-producing pathologic antibodies with serous macular detachment being an uncommon ocular manifestation. We ascertained the clinical course of maculopathy in paraproteinemia and investigated the effect of various therapeutic methods on the resolution of subretinal deposits.Design: Multicenter, retrospective, observational case series.Participants: The records of patients with paraproteinemia with optical coherence tomography (OCT) documentation of serous macular detachment were reviewed.Methods: Data collection included coexisting morbidity, rheology data (immunoglobulin level, hematocrit, and blood viscosity), clinical examination results, and OCT findings.Main Outcome Measures: Best-corrected visual acuity (BCVA), height and basal area of the serous macular detachment, and systemic versus local therapies.Results: A total of 33 cases were collected: 10 new and 23 previously reported in the literature. Diabetes was present in 7 patients, systemic hypertension in 9 patients, and anemia in 18. Mean initial immunoglobulin level was 6497 mg/dl, and mean serum viscosity was 5.5 centipoise (cP). Mean logarithm of the minimum angle of resolution initial vs. final BCVA was 0.55 (Snellen equivalent, 20/71) vs. 0.45 (20/56) in the right eye and 0.38 (20/48) vs. 0.50 (20/63) in the left eye. After mean follow-up of 7 months (range, 0-51 months). Systemic therapies included plasmapheresis (18), chemotherapy (30), blood transfusions (2), transplantation of progenitor hematopoietic cells (2), and oral rituximab (10). Immunoglobulin levels normalized in 8 patients and were unchanged in 1 after plasmapheresis, chemotherapy, or both. Ocular therapy in 8 patients included vitrectomy (1), laser photocoagulation (4), intravitreal bevacizumab (5), intravitreal triamcinolone (2), intravitreal dexamethasone implant (1), intravitreal rituximab (1), and sub-Tenon corticosteroid (1). The maculopathy resolved partially or completely in 17 patients and worsened or remained unchanged in 14 patients over median follow-up of 7 months. Maculopathy was unilateral in 9 cases and occurred at a lower initial immunoglobulin level in diabetics. There was a positive correlation between area of the detachment and serum viscosity.Conclusions: Paraproteinemicmaculopathy can be unilateral. Decreasing the blood immunoglobulin level is the primary goal of therapy for paraproteinemic maculopathy, and this can be achieved by a systemic route. Coexisting diabetes facilitates leakage of immunoglobulins at lower levels than in nondiabetics.

AB - Purpose: Paraproteinemia relates to monoclonal gammopathy-producing pathologic antibodies with serous macular detachment being an uncommon ocular manifestation. We ascertained the clinical course of maculopathy in paraproteinemia and investigated the effect of various therapeutic methods on the resolution of subretinal deposits.Design: Multicenter, retrospective, observational case series.Participants: The records of patients with paraproteinemia with optical coherence tomography (OCT) documentation of serous macular detachment were reviewed.Methods: Data collection included coexisting morbidity, rheology data (immunoglobulin level, hematocrit, and blood viscosity), clinical examination results, and OCT findings.Main Outcome Measures: Best-corrected visual acuity (BCVA), height and basal area of the serous macular detachment, and systemic versus local therapies.Results: A total of 33 cases were collected: 10 new and 23 previously reported in the literature. Diabetes was present in 7 patients, systemic hypertension in 9 patients, and anemia in 18. Mean initial immunoglobulin level was 6497 mg/dl, and mean serum viscosity was 5.5 centipoise (cP). Mean logarithm of the minimum angle of resolution initial vs. final BCVA was 0.55 (Snellen equivalent, 20/71) vs. 0.45 (20/56) in the right eye and 0.38 (20/48) vs. 0.50 (20/63) in the left eye. After mean follow-up of 7 months (range, 0-51 months). Systemic therapies included plasmapheresis (18), chemotherapy (30), blood transfusions (2), transplantation of progenitor hematopoietic cells (2), and oral rituximab (10). Immunoglobulin levels normalized in 8 patients and were unchanged in 1 after plasmapheresis, chemotherapy, or both. Ocular therapy in 8 patients included vitrectomy (1), laser photocoagulation (4), intravitreal bevacizumab (5), intravitreal triamcinolone (2), intravitreal dexamethasone implant (1), intravitreal rituximab (1), and sub-Tenon corticosteroid (1). The maculopathy resolved partially or completely in 17 patients and worsened or remained unchanged in 14 patients over median follow-up of 7 months. Maculopathy was unilateral in 9 cases and occurred at a lower initial immunoglobulin level in diabetics. There was a positive correlation between area of the detachment and serum viscosity.Conclusions: Paraproteinemicmaculopathy can be unilateral. Decreasing the blood immunoglobulin level is the primary goal of therapy for paraproteinemic maculopathy, and this can be achieved by a systemic route. Coexisting diabetes facilitates leakage of immunoglobulins at lower levels than in nondiabetics.

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