Good Pasture Syndrome- A Case Presentation

 

Mr. Moses Kandula, Mrs. P. Karthika, Ms. Nadira

Principal, Medical - Surgical (Critical Care Nursing), Welfare Institute of Nursing and Midwifery, Bharuch

Associate Professor, Child Health Nursing, Welfare Institute of Nursing and Midwifery, Bharuch, Gujarat

B Sc Nursing III Year Student, Welfare College of Nursing and Midwifery, Bharuch, Gujarat

*Corresponding Author E-mail: karthinsg473@gmail.com

 

ABSTRACT:

Good pasture syndrome (GPS), also known as Pneumorenal Syndrome or anti-glomerular basement membrane disease, is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs and kidney failure. Currently, the preferred term for both conditions is “anti-GBM antibody disease. Circulating antibodies are directed against the collagen of the part of the kidney known as the glomerular basement membrane (GBM), leading to bleeding from the lungs and kidney failure. Antibodies also attack the collagen of the air sacs of the lung (alveoli) resulting in bleeding of the lung (pulmonary hemorrhage). Good pasture syndrome is fatal unless quickly diagnosed and treated. (Magalini SI, et al, 1997)

 

KEYWORDS: Early recognition, glomerular basement membrane, kidney failure.

 

 


INTRODUCTION:

General Discussion:

Good pasture syndrome is a rare autoimmune disorder characterized by inflammation of the filtering structures (glomeruli) of the kidneys (glomerulonephritis) and excessive bleeding into the lungs (pulmonary hemorrhaging). Autoimmune syndromes occur when the body's natural defenses (antibodies) against invading or "foreign" organisms begin to attack the body's own tissue, often for unknown reasons. Symptoms of Good pasture syndrome include recurrent episodes of coughing up of blood (hemoptysis), difficulty breathing (dyspnea), fatigue, chest pain, and/or abnormally low levels of circulating red blood cells (anemia). In many cases, Good pasture syndrome may result in an inability of the kidneys to process waste products from the blood and excrete them in the urine (acute renal failure).

 

 

In some cases of Good pasture syndrome, affected individuals have had an upper respiratory tract infection before the development of the disorder. The exact cause of Good pasture syndrome is not known. In Good pasture syndrome, immune cells produce antibodies against a specific region of collagen. The antibodies attack the collagen in the lungs and kidneys. Ernest Good pasture first described the syndrome during the influenza pandemic of 1919 when he reported on a patient who died from bleeding in the lungs and kidney failure. (Brenner BM, 1997)

 

Good pasture syndrome includes all of the following conditions:

·       Glomerulonephritis—inflammation of the glomeruli, which are tiny clusters of looping blood vessels in the kidneys that help filter wastes and extra water from the blood

·       The presence of anti-glomerular basement membrane (GBM) antibodies; the GBM is part of the glomeruli and is composed of collagen and other proteins

·       Bleeding in the lungs. (Fishman AP, 1998)

 

 

Epidemiology: (Kelly PT, 2004):

Ÿ  It affects males more frequently than females.

Ÿ  Age of onset is usually between 20 and 30, but individuals at any age may be affected.

Ÿ  Men are 8 times more likely to be affected than females

Ÿ  It common in early adulthood

Ÿ  Lives in high altitude

 

Etiology: (Levy JB, 2005):

Ÿ  Unknown etiology

Ÿ  Autoimmune disorder.

Ÿ  Insult (injury) to the pulmonary vasculature

Ÿ  Viral respiratory infection

Ÿ  Genetic disorder

Ÿ  Cocaine inhalation

Ÿ  Bacteremia

Ÿ  Sepsis

Ÿ  Smoking

Ÿ  Use of hair dyes

Ÿ  Exposure to hydrocarbon fumes, metallic dust, and certain drugs, such as cocaine

Ÿ  Pulmonary hemorrhage

Ÿ  High oxygen environment

Ÿ  Anti lymphocytic treatment

 

Symptoms: (Moreso F, 2008):

The signs and symptoms related to the lung disease may include:

Ÿ  Bleeding from the nose (hemoptysis), which occur before the kidney disease in about two thirds of cases and is present in 82%-90% of the adults.

Ÿ  Cough (40%-60% of the cases)

Ÿ  Breathing difficulty (dyspnea) in about 57%-72% of the cases

Ÿ  Pallor (the most common clinical sign)

Ÿ  Crackles and rhonchi (low-pitched, rattling sound)

Ÿ  Heart murmur (20-25% of the cases)

Ÿ  Enlarged liver (hepatomegaly)

Ÿ  Edema

 

The signs and symptoms related to the kidney disease may include:

Ÿ  Blood in urine (hematuria)

Ÿ  Protein in urine (proteinuria)

Ÿ  Abnormal kidney function

Ÿ  Hypertension (high blood pressure) can be present but is not very common (reported in 4-17% of adult patients and very rare in children)

 

Confirmative diagnosis:

·       A diagnosis of anti-GBM antibody disease is made when a patient presents with lung hemorrhage, urinary findings such as proteinuria (protein in the urine) and hematuria (blood in the urine), and circulating anti–glomerular basement membrane (anti-GBM) antibodies

·       A kidney biopsy is the best method for detecting anti-GBM antibodies in tissues.

·       Light microscopy usually shows a feature known as crescentic glomerulonephritis, whereas immunofluorescence microscopy demonstrates a finding that is characteristic of this disease, of “linear deposition of IgG along the glomerular capillaries.

 

Management: (Kalluri R, 2016):

The treatment of choice is Plasmapheresis in conjunction with prednisone and cyclophosphamide. The three main goals for the treatment are:

1.     Rapidly remove circulating antibody, primarily by plasmapheresis.

2.     Stop further production of antibodies using immunosuppressant with medications, namely, corticosteroids (e.g., prednisone) and cyclophosphamide. In children, plasmapheresis is done together with corticosteroids and cyclophosphamide. The duration of the immunosuppressive treatment varies but is typically 6 months for corticosteroids and 3 months for cyclophosphamide.

3.     Remove offending agents that may have initiated the antibody production.

 

After hospital discharge, patients require long-term regular visits for monitoring kidney function and for immunosuppressive therapy. If kidney function does not return, dialysis is continued indefinitely and the patient should be referred for kidney transplantation.

 

Survival rate of Good pasture syndrome:

Good pasture syndrome was usually fatal. Aggressive therapy with plasmapheresis, corticosteroids, and immunosuppressive agents has dramatically improved prognosis. With this approach, the 5-year survival rate exceeds 80% and fewer than 30% of patients require long-term dialysis.

 

Is Good pasture's syndrome curable?

If caught early, Good pasture's Syndrome can be cured. Medications and blood treatments are used. ... There are usually no long lasting effects to the lungs, but if Good pasture's Syndrome is left untreated, bleeding and other complications can lead to death.

 

Is Good pasture syndrome contagious?

The body's immune system produces antibodies, which are proteins that help fight infections. However in Good pasture's Syndrome the body makes antibodies that attack and damage the lining of your lungs and kidney.

 

It is not contagious and it is more common in men and Caucasians.

 

A CASE REPORT:

A young child of 14 years old boy has diagnosed with Good pasture syndrome. Since the chest pain, coughing, swelling on face and extremities, weight loss day by day, yellow sclera, edema present over eyes, dry buccal mucosa, use of accessory muscles and audible wheezing sound presents during respiration and also fluid collection in both the lungs, when comes to cardiovascular status rapid pulse rate of 92 beats per min, high pitched sound, shortness of breath, hepatomegaly and burning micturation present. Due to his illness and the treatment has to be done emergency child got admitted in PICU.

 

By assessing the young child doctor has advised for investigations to conform the diagnosis. The child reports are HB – 6.2gm/dl, total RBC – 3.3mil/cu mm, PCV- 24.6%, WBC – 22000/cu mm, platelets Count.- 3, 32000/cu mm, serum creatinine is elevated. Total protein 3.0gm/dl, total bilirubin – 0.5mg/dl, blood urea 105.07mg/dl, CRP- 103.18mg/dl on urine examination colour- pale yellow, PH- 5.0,  protein 3+, hematuria 3+ , Epithelial cells 7.8/HPF, pus cells 9-10/HPF, granular cast +, blood USG – mild hepatomegaly, chest USG mild effusion volume around 100cc.

 

Than I was so afraid of all the symptoms how does the young child in suffering in schooling period. By his persistent, typical symptoms and through the support of investigations doctor has diagnosed as good pasture syndrome.

 

I felt sympathy towards the young child in his early childhood how much pain and agony that he undergone in this days was unbearable. Bt he can overcome this struggle with health worker support and prayers who is caring for this young child. Than I could realize that the life is perishable and nothing. A life without sorrows and problems we could not enjoy the happiness of life in its full measure.

 

The only fact which was consoling is removal of sputum secretions through ICD and also through support of dialysis.

 

This is very urgent, emergency condition that necessary action has to be carried out in a very specific manner. Doctors are in the treatment part such as Inj. xone 1(1gm), ceftriaxone sodium 10cc/bd/iv, Inj. vancomycine 1gm/7.7cc/Bd, Tab. Azithromycin OD, ipecac syrup oral Bd, Inj tazotum 1gm/od, Inj tramadol 2ml/tds, inj lasix 2cc/ state, tab. linezolid ½ Bd, Inj. Pan sec 40gm iv Bd, syrup potchlor 10ml/OD. If condition is not treated with supportive drugs dialysis, plasma pharesis, corticosteroids and immuno suppressive agent has to be started.

 

It is very hard to overcome all this symptoms. But through the support of doctors and other healthcare workers and grace of god we can treat this worsening situation.

 

CONCLUSION:

I have understood that how the young child is suffering in his early childhood through the Good pasture syndrome. If delay to start treatment the condition can be more fatal as it can end the child’s life too. Then the child dreams and family desires will be shattered. It’s our duty that all the health care workers and parents are responsible in treating this young child. Let’s save the young child who is in life threatening condition. Let’s join our hands together to support these young children in saving their life. Knowledge of Good Pasture Syndrome will help us to act promptly in identifying and treating the above discussed cases as a Nurse

 

REFERENCES:

BOOK REFERENCE:

1.        Magalini SI, et al., eds. Dictionary of Medical Syndromes. 4th ed. New York, NY: Lippincott-Raven Publishers; 1997:330-31.

2.        Stein JH, et al., eds. Internal Medicine, 4th Ed..: Mosby-Year Book, Inc.; 1994:2706.

3.        Brenner BM, et al., eds. The Kidney, 4th ed. Philadelphia, PA: W. B. Saunders Company; 1991:1301-05.

4.        Fishman AP, ed. Pulmonary Diseases and Disorders, 2nd ed. New York, NY: McGraw-Hill Book Company; 1988:675-81.

5.        Shah MK, Hugghins SY, Characteristics and outcomes of patients with Goodpasture’s syndrome. South Med J. 2002; 95:1411-8.

6.        Shah MK. Outcomes in patients with Goodpature’s syndrome and hydrocarbon exposure. Ren Fail. 2002; 24:545-55.

7.        Lettiere C, Pina J. Goodpasture’s syndrome: a case of delayed appearance of autoantibodies and renal disease. Mil Med. 2001; 166:827-30.

8.        Levy JB, et al. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med. 2001; 134:1033-42.

9.        Laczika K, et al. Immunoadsorption in Goodpasture’s syndrome. Am J Kidney Dis. 2000; 36:392-95.

10.      Von Vigier RO, et al. Pulmonary renal syndrome in childhood: a report of twenty-one cases and a review of the literature. Pediatr Pulmonol. 2000; 29:382-88.

11.      Phelps RG, Rees AJ. The HLA complex in Goodpasture’s syndrome: a model for analyzing susceptibility to autoimmunity. Kidney Int. 1999; 56:1638-53.

12.      Levy JB, et al. Recurrent Goodpasture’s disease. Am J Kidney Dis. 1996; 27:573-78.

13.      Turner AN, et al. Goodpasture’s disease and Alport’s syndromes. Annu Rev Med. 1996; 47:377-86.

14.      Kalluri R, et al. Specificity of circulating and tissue-bound autoantibodies in Goodpasture syndrome. Proc Assoc Am Physicians. 1996; 108:134-39.

15.      Molinier S, et al. Goodpasture syndrome: role of an epidemiological factor? Apropos of two cases. Rev Med Interne. 1995; 16:589-94.

16.      Izquierdo PM, et al. Goodpasture’s syndrome with negative renal immunofluorescence. Med Clin (Barc). 1996; 106:784-86.

17.      Moreso F, et al. Therapeutic immunoadsorption in Goodpasture disease. Med Clin (Barc). 1995; 105:59-61.

18.      Kelly PT, et al. Goodpasture syndrome: molecular and clinical advances. Medicine (Baltimore). 1994; 73:171-85.

19.      McCarthy LJ, et al. Good pasture’s syndrome in childhood: treatment with plasmapheresis and immune suppression. J Clin Aphaeresis’. 1994; 9:116-19.

20.      Liebert A, et al. Immunomodulation with apheresis technics. Allerg Immune (Leipz). 1986; 32:5-18.

21.      Marcandoro J, et al. Good pasture’s syndrome: development of its prognosis from 1955 to 1982. Presse Med. 1985; 12:1483-87.

22.      Holdsworth S, et al. The clinical spectrum of acute glomerulonephritis and lung haemorrhage (Good pasture’s syndrome). Q J Med. 1985; 55: 75-86.

23.      Medscape Reference. March 5, 2015; http://www.emedicine.com/ped/TOPIC888.HTM

24.      Medscape Reference. 2016; http://emedicine.medscape.com/article/240556-

25.      Anti-Glomerular Basement Membrane Disease. Medical Subject Headings. 2008; https://id.nlm.nih.gov/mesh/D019867.html.

26.      Pusey CD & Kalluri R. Pathogenesis and diagnosis of anti-GBM antibody (Good pasture's) disease. UpT oDate. January 13, 2016; http://www.uptodate.com/contents/pathogenesis-and-diagnosis-of-anti-gbm-antibody-goodpastures-disease.

27.      Goodpasture syndrome. Medline Plus. 9/22/2015; http://www.nlm.nih.gov/medlineplus/ency/article/000142.htm.

28.      Stem Cell Transplant for Cancer. American Cancer Society. 5/11/2016; http://www.cancer.org/acs/groups/cid/documents/webcontent/003215-pdf.pdf.

 

 

 

 

Received on 09.04.2019          Modified on 01.06.2019

Accepted on 24.06.2019     © A&V Publications all right reserved

Int. J. Nur. Edu. and Research. 2019; 7(3):408-411.  

DOI: 10.5958/2454-2660.2019.00092.9