title: Paraproteinemic maculopathy.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  Multicenter, retrospective, observational case series.
  
  
  METHODS
  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.
  
  
  METHODS
  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
  Paraproteinemic maculopathy 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.
authors.affiliation: Departments of Ophthalmology, American University of Beirut and Rafic Hariri University Hospital, Beirut, Lebanon. Electronic address: ammansourmd@gmail.com.
authors.forename: Ahmad M
authors.initials: AM
authors.lastname: Mansour
authors.affiliation: Retina Departments, The King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia, and Wilmer Eye Institute, The Johns Hopkins University, Baltimore, Maryland.
authors.forename: J Fernando
authors.initials: JF
authors.lastname: Arevalo
authors.affiliation: Department of Ophthalmology, Hospital Moises Broggi Sant Joan Despi, Barcelona, Spain.
authors.forename: Josep
authors.initials: J
authors.lastname: Badal
authors.affiliation: Associated Vitreoretinal and Uveitis Consultants, Department of Ophthalmology, Indiana University School of Medicine, Indianapolis, Indiana.
authors.forename: Ramana S
authors.initials: RS
authors.lastname: Moorthy
authors.affiliation: The Retina Institute, St. Louis, Missouri.
authors.forename: Gaurav K
authors.initials: GK
authors.lastname: Shah
authors.affiliation: Retina Associates of Cleveland, Beachwood, Ohio.
authors.forename: Hernando
authors.initials: H
authors.lastname: Zegarra
authors.affiliation: Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Jose S
authors.initials: JS
authors.lastname: Pulido
authors.affiliation: Department of Statistics and Research Methodology, Lebanese American University and Lebanese University, Beirut, Lebanon.
authors.forename: Abdulrazzak
authors.initials: A
authors.lastname: Charbaji
authors.affiliation: Department of Ophthalmology, Hospital Moises Broggi Sant Joan Despi, Barcelona, Spain.
authors.forename: Luis
authors.initials: L
authors.lastname: Amselem
authors.affiliation: Retina Service at Oftalmologica, San Miguel de Tucumán, Argentina.
authors.forename: Alejandro Jose
authors.initials: AJ
authors.lastname: Lavaque
authors.affiliation: Department of Ophthalmology, Universidade Estadual de Londrina, Parana, Brazil.
authors.forename: Antonio
authors.initials: A
authors.lastname: Casella
authors.affiliation: The Retina Institute, St. Louis, Missouri.
authors.forename: Baseer
authors.initials: B
authors.lastname: Ahmad
authors.affiliation: Associated Vitreoretinal and Uveitis Consultants, Department of Ophthalmology, Indiana University School of Medicine, Indianapolis, Indiana.
authors.forename: Joshua G
authors.initials: JG
authors.lastname: Paschall
authors.affiliation: Department of Clinical Science "Luigi Sacco," Sacco Hospital, University of Milan, Milan, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Caimi
authors.affiliation: Department of Clinical Science "Luigi Sacco," Sacco Hospital, University of Milan, Milan, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Staurenghi
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008268:Macular Degeneration / Q000209:etiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications*; D012163:Retinal Detachment / Q000209:etiology* / Q000473:pathology; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence; D014792:Visual Acuity