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Year : 1984  |  Volume : 32  |  Issue : 5  |  Page : 325-332

Ocular response in systemic immune-deficiency states

Jaslok Hospital and Research Centre- Mumbai, India

Correspondence Address:
Kanti Mody
Jaslok Hospital & Research Centre, Mumbai
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Source of Support: None, Conflict of Interest: None

PMID: 6545315

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How to cite this article:
Mody K. Ocular response in systemic immune-deficiency states. Indian J Ophthalmol 1984;32:325-32

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Mody K. Ocular response in systemic immune-deficiency states. Indian J Ophthalmol [serial online] 1984 [cited 2023 Jun 10];32:325-32. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1984/32/5/325/27503

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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 Top

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 Top

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.


-Corneal ulcer-Pseudomonas+Staph.


-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


Visual fields Mixed

showed representation

  Discussion Top

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.


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.


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 Top

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 Top

Fedukowicz H., 1977. "External Infection of the Eye", Appleton Century-Crofts. New York.  Back to cited text no. 1
Grayson H., 1977. "Diseases of the Cornea "(1st edi­tion) Pages 39-41 The C.V. Mosby Co. St. Louis Toronto, London.  Back to cited text no. 2
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.  Back to cited text no. 3
Rahi A.H.S. and Garner A., 1976. Immunopathology of the Eye, 1st edition-Blackwell Scientific Publica­tion Oxford, London, Edinburg, Melbourne.  Back to cited text no. 4
Walter J.B. and Israel M.S., 1979. General Pathol­ogy, 5th edition, chapter 7,8,12 & 13. ,Churchill, Living­stone, London.  Back to cited text no. 5


  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]

  [Table - 1], [Table - 2]


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