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| ORIGINAL ARTICLE |
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| Year : 1984 | Volume
: 32
| Issue : 5 | Page : 325-332 |
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Ocular response in systemic immune-deficiency states
Kanti Mody
Jaslok Hospital and Research Centre- Mumbai, India
Correspondence Address:
Kanti Mody
Jaslok Hospital & Research Centre, Mumbai
India
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 6545315 
How to cite this article:
Mody K. Ocular response in systemic immune-deficiency states. Indian J Ophthalmol 1984;32:325-32 |
With the beneficial and fast developing progress in various disciplines of Medicine, there are certain areas where there has been a considerable rise in the incidence of "The Opportunistic Infection". While success (though not complete) has been achieved in the control of infection due to pathogenic organisms, now we are facing the challenging situations of the ocular involvement with and in response to "The opportunistic Infection" in different Systemic Immune Deficient (I-D) States, and conditions predisposing to I-Dstate. Occasionally these situations are frustrating and met with disappointments particularly when they are recognised late or not at all or the organisms involved get the upper hand due to lack of appropriate available therapeutic measures.
| Materials and methods | |  |
1. Clinical observations were made and these are reported in the form of a selected case study encompassing patients with I.D. state & the opportunistic infections.
2. Clinical Ophthalmic Examination were done.
3. Study of(a) The patient's general condition as well as specific condition. (b) Reports of various investigations depending on the patient's general and ocular condition including microbiology.
4. Systemic and Ocular management and its response.
| Observations | | |
Ocular response in terms of infection, inflammations and damaging effects on the eye and adjacent tissues were graded as:
mild, moderate and severe and the responses to treatment were graded. Different Associated Systemic Immune-Deficiency States [Table - 1] and different organisms (opportunistic infection) detected are tabulated.
[Table - 2]. Systemic involvement by the Opportunistic Infection, cases are described as under:
Case Study (Selected Patients)
1. Candida and Mucormycosis: [Figure - 1]
-Male 56 years old, Uncontrolled Diabetes
-Pyogenic Meningitis treated with Systemic Antibiotics
-Opportunistic infection by Candida and Mycormycosis (portal of entry-Nose).
-Involving Base of the Brain
-Multiple Cranial Nerve Pasly (Hemibase Syndrome) including II, IV, V, VI, VII)
-Treated with antifungal agents-Patient survived and doing well.
-Corneal Ulcer, Panophthalmitis, prqptosis
-CSF, Sputum, Nasal discharge, swab from corneal ulcer, ocular material after evisceration-showed Candida and Mucormycosis.
-Eye lost.
2. Long Term Steroids :
Male 32 years old patient suffering from Renal condition and on high dose long term steroids. Corneal bacterial ulcer, mild, relatively quite, complicating into panophthalmitis. Patient dies of Renal failure and septicaemia.
3. Long Term Steroids:
Male 16 years, Glomerulonephritis Malnourished. On long term high dose Systemic Steroids.
Miliary Tuberculosis Choroid Tubercle. [Figure - 2]
Died of Renal failure and Miliary Tuberculosis.
4. Glomerulonephritis on Long Term Steroids
Male 42 years.
Mild Staph. Aureus meningitis. Frontal lobe abscess, Frontal sinusitis. Orbital cellulitis extending upto superior and inferior orbital fissures.
Corneal ulcer with panophthalmitis III, IV, V, VI, VII Palsy.
Destruction of frontal bone wall (Osteomyelitis) Maggots. Patient Survived with immobile Eye. Corneal Scarring, and Reduced Vision.
5. Patients on Immunosuppression Therapy
Renal Transplant and Cancer Patient on Chemotherapy/Radiotherapy Variety of infections of the eye Fungus, Virus and Bacteria. [Figure - 3]
6. Renal Transplant on Immunosuppression
Harpes Simplex [Figure - 4]
Dendritic Ulcer of Cornea-responded to the treatment after long time.
7. Leukaemia
7 years old girl.
Herpes Zoster Ophthalmicus. Recovered-long duration.
8. Acute Lymphoblastic Leukaemia
Female baby, 1 year old.
On Chemotherapy, Low Ig. Disorder of B+T cell Immune response. High fever, nasal discharge.
Unilateral edema lids+Chernosis and congestion-orbital cellulitis No response to Four Board Spectrum Antibiotics, Long duration of Acute inflammation. X-ray showed Sinusitis. Eventually, patient survived, sinusitis and Orbital cellulitis settle well.
9. Multiple Myeloma and Leukaemia
a) 48 years female
-Sneezing-Surgical emphysema in the orbit.
--Infection-severe Chemosis
-Slow response to Local and Systemic Antibiotics.
-Eventual recovery of the eye condition with minimal corneal scar.
b) -Presentation with Herpes Zoster Ophthalmicus and VI Nerve Paresis.
c) -Inflammatory Response in the form of Orbital Cellulitis following Radiotherapy for suspected Myeloma deposits in the Orbit
10. Drug-Indubced Agranulocytosis
-18 years male, semiconscious, patient with neurological disorder and Systemic infection.
-Given Systemic Chloromycetin.
-Agranulocytosis.
-Corneal ulcer-Pseudomonas+Staph.
Aureus.
-Chloromycetin discontinued.
Patient died of septicaemia.
11. Burns
20 years old female
-Extensive bums, Muscle involvement. -Pseudomonas infection in the muscles -Corneal ulcer with pseudomonas followed by perforation
-Died of septicaemia and shock.
12. Diabetes
-Uncontrolled, poor resistance
-Lack of adequate nutrition liable to get infection (Bacterial and Fungus) Involving the eye.
-Depressed Reactivity of Human Lymphocytes to Phytohaemagglutination corrected after therapy.
-Impaired PMN Chemotaxis in Ketosis and poorly controlled Diabetes Mellitus.
13. Pseudo-conjunctivitis
-26 years old male with Renal Transplant
-Recurrent Conjunctivities
-No response to local/systemic antibiotics
-Dramatic resolution whenever he was bled for resultant Polycythaemia.
-Polycythaemia gives a false appearance of conjunctivities.
14. Dry Eye Syndrome
-Sjogren's Syndrome
-Refsum's Disease
-Recurrent Corneal Ulcers
15. Old age, Malnutrition, Semiconscious, Unconscious State Absent slow Blink Reflex, Leading to Corneal Ulcer.
16. -Malnourished child with protein calories deficiency
-Corneal ulcer
-Vitamin A deficiency-Xerophthalmia, Corneal Ulcer, Keratomalacia.
17. Ataxia Telangiectasia [Figure - 5]
Autosomal Recessive
Cerebellar Ataxia-progressive
Oculocutaneous Telangiectasia
Recurrent Sinus and Pulmonary infection (Virus-Bacteria)
Thymus activity is depressed
Delayed type of reaction-absent or inadequate, Lower levels of IgA and E.
18. Vitreous Heamorrhage Following Bee-Bite
18 years male - on Mountain hiking
-On returning at night fell down on a Bee-Hive
-Severe Bee-Bite-Unconscious -Hospitalised
-Acute Renal Failure
-Disseminated Intravascular Coagulation-Bleeding
-Vitreous Haemorrhage
-There are some doubts and different views as to whether the above case can be considered as an I-D State.
19 Renal Transplant Immunosuppression and long term steroids
43 years Female
Renal Transplant on Immuno Suppression and Long Term Steroids.
Pulmonary Th Raised I.O.P.
Rx-Ethambutol for a length of
Toxicity time
Temporal Pallor Optic Atrophy
(cupping)
Visual fields Mixed
showed representation
| Discussion | | |
Immunity is the ability to resist or overcome infection. It depends on (1) specific immunity (Immune Response) (2) Nonspecific Immunity and (3) Virulence of the organism. The Immune Resistance of the animal and Virulence of the organisms are alternative ways of considering the relationship as well as the outcome between the host and the parasite.
If Resistance/Response be suppressed in certain situations, it can lead to severe infection by organisms of low virulence which is known as "The Opportunistic Infection .
Non-specific Immunity depends on:
a) Previous contact
b) Humoral factors
c) Cellular factors and
d) Genetic factors consisting of species, racial and individual responses.
Specific Immunity is related to a specific Immune Response to the Organisms. In I-D or Immunologically compromised state, Non-specific Immunity is depressed, suppressed, inadequate, absent, abnormal or congenitally defective. Reduced Polymorphs, Qualitative defects in the Polymorph in the form of abnormality of chemotaxis, adherence to bacteria, phagocytosis, random movement, intracellular bactericidal activity, increased susceptibility to infection etc. contribute to Immune deficiency.
There are other factors associated with impaired bacterial killing e.g. various enzyme deficiency eg. Glucose - 6 phosphate Dehydrogenase deficiency, myeloperoxidase deficiency and pyruvate kinase deficiency. Neutrophil function is known to be adversely affected in Diabetes Mellitus, Cirrhosis of Liver, Hodgkin's disease, administration of drugs (steroids, phenylbutazone and alcohol). In some I-D state there is a deficiency of complement components, anaphylatoxins and chemotactic factors are defective, defects in the Monocytes and RE. System as well as defects in complements. Tlymphocyte and B-lymphocyte function or their formation may be defective.
In addition, stem cell deficiency, Thymic deficiency. Immunoglobulin deficiency and mixed deficiency are well known in congenitally Immuno-deficient diseases. (Ataxia Telangiectasia)
Natural Defence Mechanism in the Eye and its Breakdown
Intact skin and mucous membrane form an important first line of defence against any infection. Corneal epithelium is a natural barrier to bacterial and fungal organisms (except t6N. Gonorrhoeae, Corynebacterium Diphtheriae and Listeria species). Blink reflex, mechanical flushing action of tears, presence of lysozyme, Betalysins and host Phagocytosis, specific humoral and cellular reactions etc. defend the host and also take care of opportunistic organisms.
Corneal abrasion, exposure, erosion, foreign body etc. may precede the corneal ulcer which is a great potential source of danger in an individual with immune deficient state (or Immunologically compromised host) e.g. debilitated, elderly, alcoholic malnutrition, Diabetes mellitus, Sjogren's syndrome etc.
Transplant Patients, Immuno Suppression and Cancer Patients on Radio/Chemotherapy
The above group of patients are always at a great risk and very susceptible to the opportunistic infection, particularly the drugs and radiotherapy tend to decrease Immune mechanism and lead to the compromised state. Steroids, Immuno-suppressive agents, Cytotoxic agents e.g. Azathioprine, folic acid Antagonists and Alkylating agents etc. predispose the patients to the opportunistic infections.
Antibiotics
Broad spectrum antibiotics alter the normal flora of the host, thereby facilitating the overgrowth of resistant pathogens. Abuse of the antibiotics contributes to a compromising situation by abnormal gram-negative flora.
In some cases, the antibiotics lead to Bonemarrow depression or to agranulocytosis, neutropenia etc. thus favouring the opportunistic infection.
New Born, Elderly, Malnutrition etc.
In the new born, the cellular immune system is slow to develop while in the old age group there is a decline in the ability of the body to react to the opportunistic infection by inflammatory and cellular mechanisms. Malnutrition, debilitating diseases. drug addiction, alcoholism etc. are predisposing conditions to the opportunistic infection particularly after formation of corneal ulcer following corneal abrasion, exposure, absent or slow blink reflex, erosion, foreign body etc.
In Vitamin A deficiency, Corneal Epithelial barrier is not intact leading to ulceration, Keratomalacia etc. While in protein-calory deficiency, cellular immunity is suppressed.
Blood Diseases
In chronic lymphatic leukaemia there is depression of Immunoglobulin and cell bound antibody. In multiple myeloma there is an impairment of normal immunoglobulin production. In Acute Lymphoblastic Leukaemia there is low level of Serum Ig, associated disorders of B and T cells immune response. These patients are on Chemotherapy (as well as Radiotherapy).
Dry Eye Syndromes
In systemic Disease with dry eye (Sjogren's Syndrome) there is a lack of tears and its protective components as well as protective layer on the cornea. This is liable to form an ulcer with invasion of opportunistic organisms.
In Burns and Crush Injuries Involving Muscles
There is a tendency to contact pseudomonas infection which is very likely to involve the eye.
Indwelling cannulae, I.V. therapy, I.V. Narcotic abuse etc. provide unsterile sources of entry to opportunistic organisms. Surgical wounds and Trauma are other examples of predisposing conditions.
Diabetes
In poorly controlled Diabetes, there is depressed reactivity to PHA (Phytohaemagglutination). This can be corrected by Insulin therapy. An impairment in Polymorphonuclean Cemotaxis is observed in ketotic state and poorly controlled Diabetes.
Congenital Immuno Deficiency Disease
It consists of various diseases depending on deficiency syndrome and the syndromes of . mixed deficiencies e.g. Ataxia Telangiectasia.
| Summary | | |
In view of the increasing incidence of the Opportunistic Infection in Systemic Immune Deficiency States and in the conditions predisposing it, it is essential for the Ophthalmologist to be aware of the Ocular Response in these situations.
Unusual, exaggerated Ocular Responses in various Systemic Immune- Deficiency states were observed in a study carried out of Jaslok Hospital and research centre on 50 patients. Immune Deficiency states included Organ Transplant, Steroids, Chemo-therapy, Immune-suppression therapy, Leukaemia, infants and elderly, malnourished, Congenital Immune deficient disease, Diabetes, burns etc. opportunistic infection included various bacteria, viruses and fungi. The early detection, recognition, prompt diagnosis and prompt management of this Ocular Response contribute a great deal to the prevention of blindness.[5]
| References | | |
| 1. | Fedukowicz H., 1977. "External Infection of the Eye", Appleton Century-Crofts. New York.  |
| 2. | Grayson H., 1977. "Diseases of the Cornea "(1st edition) Pages 39-41 The C.V. Mosby Co. St. Louis Toronto, London. |
| 3. | Paymen G.A., Sanders D.A., Goldberg M.F., 1957, Principles and Practice of Ophthalmology (1st edition), Vol. III Pages 1654. W.B. Saunders Company, Philadelphia, London. |
| 4. | Rahi A.H.S. and Garner A., 1976. Immunopathology of the Eye, 1st edition-Blackwell Scientific Publication Oxford, London, Edinburg, Melbourne. |
| 5. | Walter J.B. and Israel M.S., 1979. General Pathology, 5th edition, chapter 7,8,12 & 13. ,Churchill, Livingstone, London. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]
[Table - 1], [Table - 2]
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