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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 21  |  Issue : 5  |  Page : 935-939
Goodpasture's syndrome in children


1 Department of Critical Care Medicine, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
2 Department of Nephrology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India

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Date of Web Publication31-Aug-2010
 

   Abstract 

Goodpasture's syndrome is an autoimmune disease that has rarely been described in children, and only 21 cases have been reported till now. Here is a case of a 9-year-old boy who was diagnosed to have Goodpasture's syndrome.

How to cite this article:
Poddar B, Singhal S, Azim A, Gulati S, Baronia A. Goodpasture's syndrome in children. Saudi J Kidney Dis Transpl 2010;21:935-9

How to cite this URL:
Poddar B, Singhal S, Azim A, Gulati S, Baronia A. Goodpasture's syndrome in children. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2019 Jul 16];21:935-9. Available from: http://www.sjkdt.org/text.asp?2010/21/5/935/68896

   Introduction Top


The term pulmonary-renal syndrome has fre­quently been used to describe the clinical mani­festations of a great number of diseases in which pulmonary hemorrhage and glomerulo­nephritis coexist. [1],[2],[3],[4] Though rare in childhood, pulmonary renal syndrome presents a medical emergency. Goodpasture's syndrome (GPS) as a cause of pulmonary-renal syndrome is rare in childhood. [5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22]


   Case Report Top


A 9-year-old boy was admitted to his local hospital with a two weeks history of low-grade fever and vomiting. There was no past or family history of renal disease. Five days later, he developed cola-colored urine. The urinalysis re­vealed protein 2(+), blood 3(+) and numerous dysmorphic red blood cells per high power field. Renal function deteriorated progressively with oligoanuria and marked uremia over the next few days for which peritoneal dialysis was started. As he was anemic, he received two units of whole blood transfusion. For further evaluation, he was referred to the department of Nephrology in our hospital, where peritoneal dialysis was continued. On admission, examina­tion revealed a pale patient with bilateral pedal edema and blood pressure of 180/100 mmHg. His hemoglobin was 7.2 g/dL, total leukocyte count 16800/mm 3 and platelets 497,000/mm 3 with normal coagulation tests. Serum biochemistry revealed blood urea nitrogen (BUN) 83 mg/dL, creatinine 8.8 gm/dL and normal liver function tests. He was seronegative for HIV and Hepatitis B and C. Chest radiograph and electrocardiogram were normal. An ultrasound of abdomen re­vealed enlarged kidneys with increased cortical echogenicity with loss of corticomedullary dif­ferentiation. While planning for a renal biopsy, the patient developed respiratory distress asso­ciated with an episode of hemoptysis. Recent chest radiograph revealed bilateral interstitial al­veolar infiltrates [Figure 1]. Hemodialysis was initiated, but respiratory distress continued. Be­cause of increasing respiratory distress, the pa­tient was transferred to the ICU where he was intubated and put on mechanical ventilation. The enzyme linked immunosorbent assay (ELISA) for perinuclear antineutrophil cytoplasm antibo­dies (p-ANCA) and anti-myeloperoxidase (anti­MPO) was positive. ELISA for anti-GBM (glo­merular basement membrane) antibodies was strongly positive (titre 1514.4 U/mL). Assays for ANCA revealed negative indirect immunofluore­scence for c-ANCA (cytoplasmic) and p-ANCA. Complements levels were elevated (C3-139 and C4-32.3 mg/dL). Pulse methylprednisolone fol­lowed by oral prednisolone, cyclophosphamide, and plasmapheresis on a daily basis were star­ted. Intermittent hemodialysis was continued and 21 sessions of plasmapheresis were completed. The blood pressure was controlled with 5 anti­hypertensive drugs (ramipril, nifedipine, propra­nolol, prazosin and clonidine). The weekly mea­sured titer of anti-GBM antibody levels started to decrease and was strongly associated with clinical improvement. The pulmonary hemo­rrhage resolved and the patient was gradually weaned off the ventilator, but his renal function did not improve and he continued to require intermittent dialysis. After weaning from venti­ lation, ambulatory peritoneal dialysis was star­ted but could not be sustained due to peritonitis not responding to local and systemic antibiotics. Eventually, he was discharged home on intermi­ttent hemodialysis, anti-hypertensive drugs and immunosuppressants, but he succumbed to his illness around two months after discharge.
Figure 1 :Chest radiograph showing bilateral interstitial alveolar infiltrates (right > left) suggestive of pulmonary hemorrhage.

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   Discussion Top


Pulmonary renal syndrome can be categorized into specific and nonspecific. Nonspecific pul­monary renal syndrome [2] refers either to pul­monary edema, thromboemblism, or infection complicating the course of glomerular disease, or to glomerular diseases following a pulmo­nary disease, mostly an infection. Though en­countered in both childhood and in adulthood, nonspecific pulmonary renal syndrome is more frequent in adults.

Specific pulmonary renal syndromes [2] are of three major categories:

  1. Goodpasture's syndrome with circulating antibodies against GBM or linear immuno­fluorescent staining of IgG in the basement membranes of pulmonary or renal tissue;
  2. Immune-complex-related glomerulonephritis, including the types associated with syste­mic lupus erythematosus, Henoch-Schonlein purpura, mixed connective-tissue disease, and cryoglobulinemia;
  3. glomerulonephritis associated with antineu­trophil cytoplasmic antibodies (ANCA), with­out immune complexes or anti-GBM anti­body, including Wegener's granulomatosis, microscopic polyarteritis, the Churg-Strauss syndrome, and classic polyarteritis nodosa.


In childhood, the most frequent causes of pul­monary renal syndrome are systemic lupus ery­thematosus, ANCA-associated systemic vascu­litis, Henoch-Scholein purpura and hemolytic­uremic syndrome. [3] Goodpasture's syndrome as a cause of pulmonary-renal syndrome in child­hood is extremely rare with only 21 cases re­ported until now in the medical literature. [4],[5],[6],[7],l[8],[9],[10],[11],[1],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22] The previously reported cases of Goodpasture's syndrome are detailed in [Table 1]. Among the 22 pediatric patients with Goodpasture's syndrome, including ours, there are six males in com­parison to 14 females with male to female ratio of 0.43, and the majority of the cases (15 out of 20) are between 6-13 years of age. In contrast to this, in adults, it is more common in males (male to female ratio = 4:1) with peak incidence between 20-30 years of age. [23] Anti-GBM titers were positive in all the patients in which it was done (16/16); out of them, only one patient showed an indeterminate result. Anti-MPO titer was positive in only four patients along with the anti-GBM titers. Our patient also had both anti­MPO and anti-GBM. Renal biopsy was diagno­stic in 12 out of 15 (80%) of the patients; biop­sy could be obtained after death in 3 patients. Pulmonary biopsy was not obtained from any pa­tient during life. In most of the patients (10/14, 71.4%), the treatment strategy consisted of ste­roid and immunosuppressants along with plas­mapheresis. In 4 patients, the diagnosis could be made only after death. Only a third of the pa­tients (5/15) showed improvement in renal func­tion, of which one became dialysis-dependent later. In patients with non improved renal func­tion, renal transplant was performed in 2/11 (18.2%) of the patients, whereas the majority (9/11, 81.8%) remained on intermittent dialysis. Six out of 18 (33.3%) patients died in compa­rison to mortality of 11-21% in adults. [24]

The diagnosis of Goodpasture's syndrome can be confirmed by the presence of circulating anti-GBM antibodies and/or deposition of anti­bodies on the glomerular basement membrane demonstrated by immunofluorescent (IF) stai­ning of the renal biopsy specimen. Pulmonary biopsy is rarely indicated because renal tissue is more easily attainable with less morbidity. [24]Management should be aggressive and started immediately upon suspicion. Therapy is based on corticosteroids; immunosuppressive agents such as cyclophasphamide in addition to plas­mapheresis. [25],[26] Renal transplantation can be per­formed in patients requiring chronic hemodia­lysis after disappearance of the circulating anti­GBM antibodies. Advanced renal failure at pre­sentation and/or presence of crescents affecting more than 50% of glomeruli is a serious prog­nostic sign. [24]
Table 1 :Previously reported cases of Goodpasture's syndrome.

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In conclusion, pulmonary-renal syndrome is a rare event in childhood. Although rare, GPS should be considered in the differential diag­nosis. Pulmonary involvement is related to acute presentation, whereas renal involvement is related to long-term morbidity. Early diag­nosis is crucial for a favorable outcome of this syndrome.

 
   References Top

1.Gallagher H, Kwan JT, Jayne DR. Pulmonary renal syndrome: a 4-year single center expe­rience. Am J Kidney Dis 2002;39:42-7.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Salant DJ. Immunopathogenesis of crescentic glomerulonephritis and lung purpura. Kidney Int 1987;32:408-25.  Back to cited text no. 2  [PUBMED]    
3.Von Vigier RO, Trummler SA, Laux-End R, Sauvain MJ, Truttmann AC, Bianchetti MG. Pulmonary renal syndrome in childhood: a report of twenty-one cases and a review of the literature. Pediatr Pulmonol 2000;29:382-8.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.O'Connell EJ, Dower JC, Burke EC, Brown AL, McCaughey WT. Pulmonary hemorrhage­glomerulonephritis syndrome. Am J Dis Child 1964;108:302-8.  Back to cited text no. 4      
5.Powell AH, Bettez PH. Goodpasture's syn­drome: pulmonary hemosiderosis with glome­rulonephritis. Can Med Assoc J 1964;90:5-9.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Ozsoylu S, Hiesonmez G, Berkel I, Burhan S, Tinaztepe B. Goodpasture's syndrome (pulmo­nary hemosiderosis with nephritis). Clin Pediatr 1976;15(4):358-60.  Back to cited text no. 6      
7.Levin M, Rigden SP, Pincott JR, Lockwood CM, Barratt TM, Dillon MJ. Goodpasture's syn­drome: treatment with plasmapheresis, immuno­suppresion, and anticoagulation. Arch Dis Child 1983;58(9):697-702.  Back to cited text no. 7      
8.Gilvarry J, Doyle GF, Gill DG. Good outcome in anti-glomerular basement membrane neph­ritis. Pediatr Nephrol 1992;6(3):244-6.  Back to cited text no. 8      
9.McCarthy LJ, Cotton J, Danielson C, Graves V, Bergstein J. Goodpasture's syndrome in child­hood: treatment with plasmapheresis and immu­nosuppresion. J Clin Apher 1994;9(2):116-9.  Back to cited text no. 9      
10.Boven K, Miljoen HP, Van Hoeck KJ, Van Marck EA, Van Acker KJ. Anti-glomerular base­ment membrane glomerulopathy in a young child. Pediatr Nephrol 1996;10:745-7.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]  
11.Bigler SA, Parry WM, Fitzwater DS, Baliga R. An 11-month old with anti-glomerular basement membrane disease. Am J Kidney Dis 1997;30(5):710-2.  Back to cited text no. 11      
12.Blanco Filho F, Ernesto LC, Rosa MA, Stuginski LA, Ziochevsky ER, Blanco F. Rapidly prog­ressive antineutrophil cytoplasm antibodies asso­ciated with pulmonary-renal syndrome in a 10­year old girl. Sao Paulo Med J 2001;119(1):29-32.  Back to cited text no. 12      
13.Hibbs AM, Bznik-Cizman B, Guttenberg M, Goldberg B, Meyers K. Ulcerative colitis in a renal transplant patient with previous Good­pasture's disease. Pediatr Nephrol 2001;16(7): 543-6.  Back to cited text no. 13      
14.Bakkaloglu SA, Kasapkara CS, Soylemezoglu O, et al. Successful management of anti-GBM disease in a 5 1/2-year old girl. Nephrol Dial Transplant 2006;21(10):2979-81.  Back to cited text no. 14      
15.Hijosa MM, Roman LE, Camblor CF, Garcia ML, Salcedo DP, Torres MN. Anti-GBM and anti-MPO antibodies in a case of pulmonary renal syndrome. Pediatr Nephrol 2005;20(6): 807-10.  Back to cited text no. 15      
16.Martini A, Binda S, Mariani G, Scotta MS, Ruberto G. Goodpasture's syndrome in a child: natural history and effect of treatment. Acta Paediatrica 1981;70(3):435-9.  Back to cited text no. 16      
17.Simonsen H, Brun C, Thomsen OF, Larsen S, Ladefoged J. Goodpasture's syndrome in twins. Acta Med Scand 1982;212(6):425-8.  Back to cited text no. 17      
18.Fortuna IaG, Russu VG, Filippova GA. Good­ pasture's syndrome in a 10-year-old girl. Paediatriia 1983;2:75-6.  Back to cited text no. 18      
19.Harrity P, Gilbert-Barness E, Cabalka A, Hong R, Zimmerman J. Isolated pulmonary Good­pasture syndrome. Paediatr Pathol 1991;11(4): 635-46.  Back to cited text no. 19      
20.Siegler RL, Bond RE, Morris AH. Treatment of Goodpasture's syndrome with plasma exchange and immunosuppresion. Clin Pediatr (Phila) 1980;19(7):488-91.  Back to cited text no. 20      
21.Rosenblum ND, Colvin RB. A 13 year-old girl with gross hematuria four years after a diagnosis of idiopathic pulmonary hemosiderosis. N Engl J Med 1993;328:1183-90.  Back to cited text no. 21      
22.Fanburg BL, Niles JL, Mark EJ. A 17 year-old girl with massive haemoptosis and acute oli­guric renal failure. N Engl J Med 1993;329: 2019-26.  Back to cited text no. 22      
23.Seaton A. Some less common pulmonary dis­orders, In: Seaton A, Seaton D, Leith AG, editors. Crofton and Douglas's Respiratory Diseases 5th ed. Oxford: Blackwell Science 2000;1330-45.  Back to cited text no. 23      
24.Salam N, Rezki H Fadili W, Hachim K, Ramdani B. Goodpasture's syndrome. Four case reports. Saudi J Kidney Dis Transpl 2007;18(2): 235-8  Back to cited text no. 24      
25.Bolton WK. Goodpasture's syndrome. Kidney Int 1996;50:1753-66.  Back to cited text no. 25  [PUBMED]    
26.Salma AD, Levy JB, Lightstone L, Pussey CD. Goodpasture's disease. Lancet 2001;358:917­-20.  Back to cited text no. 26      

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Correspondence Address:
Banani Poddar
Department of Critical Care Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow 226014
India
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