The Impact of Systemic Light Chain Amyloidosis on Eyes
Trial statusRecruiting
Trial phaseNot listed
Trial typeObservational
Biological sexAll
Age18-80
SponsorZhongshan Ophthalmic Center, Sun Yat-sen University
About this trial
Study Purpose and Principle: Amyloidosis is a group of diseases characterized by the deposition of amyloid proteins in tissues and organs throughout the body, with common affected organs including kidneys, heart, nervous system, gastrointestinal tract, and liver. Nowadays, nearly 40 different proteins have been found to form amyloid fibrils in body, among which Systemic Light Chain (AL) Amyloidosis is the most common type of systemic amyloidosis. AL amyloidosis is a plasma cell disorder, with its precursor protein originating from free light chains produced by abnormal plasma cell clones, which form amyloid substances that deposit in tissues and organs causing lesions. In terms of incidence, the incidence of AL amyloidosis is 8-10 cases per million person-years in the United States and is considered a rare disease in our country. AL amyloidosis has an insidious onset, diverse clinical manifestations, and is prone to misdiagnosis and missed diagnosis in clinical practice. The prognosis also has strong heterogeneity and is closely related to the extent of organ involvement at the time of diagnosis. In the past, the incidence of eye involvement in systemic AL amyloidosis patients was low, with various manifestations, only reported as case reports, and not regularly followed up with systemic treatment. Based on this, this project aims to assess the involvement of eyes and its appendages in patients with different stages of systemic light chain amyloidosis, with the goal of clarifying the ocular manifestations of this systemic disease and exploring early diagnostic indicators for the eye.
Primary Objective: To assess the involvement of the anterior segment of the eye, as well as the extraocular soft tissues and muscles, in patients with systemic light chain amyloidosis.
Secondary Objective: To assess the neuro-ophthalmic manifestations and changes in retinal choroidal blood flow in patients with systemic light chain amyloidosis.
Study Design: Observational study.
Study Population and Expected Enrollment: 80 patients with systemic light chain amyloidosis, 50 normal controls.
Study Duration: Six months of follow-up after the last subject is diagnosed and enrolled.
Intervention: Experimental Group: Patients with systemic light chain amyloidosis; Control Group: Subjects without systemic diseases.
Eligibility criteria
This trial accepts healthy volunteersQualifiers
Patients diagnosed with "Systemic Light Chain Amyloidosis";
No other severe systemic underlying diseases that affect the examination;
Subjects voluntarily participate in this study, sign the informed consent form;
Age between 18-80 years old, no gender restrictions.
Disqualifiers
Patients with eye diseases of clear causes such as ocular surface malignant tumors, corneal perforation, severe eye trauma, etc.;
Patients with nystagmus;
Severe ocular scar diseases; conjunctival scars with fornix shortening;
Current signs of infection, including fever and undergoing antibiotic treatment;
Trial population
Source and Selection Method of Case Group: Patients diagnosed with "Systemic Light Chain Amyloidosis" or suspected of having "Systemic Light Chain Amyloidosis" and treated according to relevant diagnostic and treatment standards at tertiary hospitals. They are planned to undergo ocular screening at Zhongshan Ophthalmic Center of Sun Yat-sen University to exclude ocular and adnexal complications. It is expected that 80 cases of systemic light chain amyloidosis will participate in this study. Source and Selection Method of Control Group: Control group subjects are all sourced from the outpatient department of Zhongshan Ophthalmic Center of Sun Yat-sen University. It is expected that 50 cases of dry eye syndrome/cataract patients will participate in this study.
Trial design
Design model
Cohort
Time perspective
Prospective
Treatments tested in this trial
Not listed
Trial groups
130 Participants
are grouped into 2 trial groupsGroup A: Exposed group
Group B: Unexposed group
Trial outcomes
Primary outcomes
1
Conjunctival Mass
Conjunctival Mass: The size of the yellowish-pink waxy mass on the bulbar or palpebral conjunctiva (if present, additional conjunctival confocal microscopy examination is performed to clarify the microscopic morphology of the mass).
Time frame
Each patient will be followed up once every 2 months, for a total of 4 follow-ups, over a 6-month period, to observe any ocular complications and progression.2
Subconjunctival Hemorrhage Grading
Subconjunctival Hemorrhage: No conjunctival congestion is scored 0; mild diffuse conjunctival congestion is scored 1; moderate diffuse conjunctival congestion, with noticeable congestion near the fornices, is scored 2; severe diffuse conjunctival congestion with subconjunctival hemorrhage is scored 3.
Time frame
Each system light chain amyloidosis patient will be followed up once every 2 months, for a total of 4 follow-ups, over a 6-month period, to observe any ocular complications and progression.Secondary outcomes
1
Periocular Purpura
Purple-red macules and papules on the skin around the orbit.
Time frame
Each system light chain amyloidosis patient will be followed up once every 2 months, for a total of 4 follow-ups, over a 6-month period, to observe any ocular complications and progression.2
Ptosis
Measure the degree to which the upper eyelid margin covers the cornea when both eyes are open and looking straight ahead. It is classified based on the degree of coverage: Mild: Coverage ≤4 mm, with a drooping amount of ≤2 mm; Moderate: Coverage \>4～≤6 mm, drooping amount \>2～≤4 mm; Severe: Coverage \>6 mm, reaching the central part of the pupil, with a drooping amount \>4 mm.
Time frame
Each patient will be followed up once every 2 months, for a total of 4 follow-ups, over a 6-month period, to observe any ocular complications and progression.3
Vitreous Opacity
Fundus photography records the morphology of vitreous opacity, such as Weiss ring, flocculent, dense membranous, fine punctate, filamentous, reticular, etc.
Time frame
Each system light chain amyloidosis patient will be followed up once every 2 months, for a total of 4 follow-ups, over a 6-month period, to observe any ocular complications and progression.4
Cup-to-Disc Ratio (C/D)
After mydriasis (pupil \> 6 mm), in a dark room, a skilled professional technician uses a digital fundus camera to adjust the machine's height, the subject's head position, and seating. The subject is instructed to open their eyes and focus on the cursor inside the machine, and two directional fundus photographs are taken for each eye (centered on the macula and the optic disc, respectively).
Time frame
Each system light chain amyloidosis patient will be followed up once every 2 months, for a total of 4 follow-ups, over a 6-month period, to observe any ocular complications and progression.Sponsors and contacts
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Source ClinicalTrials.gov