Journal of Nephrology & Renal Therapy Category: Clinical Type: Case Report
Uveitis and Late Onset Nephritis Associated With Henoch Schonlein Purpura: A Case Report
- Sare Gülfem ÖZLÜ1*, Fatma YAZILITAS2, Özlem AYDOG2, Mehmet BÜLBÜL2, Çigdem YILDIZ2, Evrim KARGIN ÇAKICI2, Gökçe ERTUGRUL3, Nilüfer ARDA4
- 1 Faculty Of Medicine, Department Of Pediatric Nephrology, Dr Sami Ulus Childrens And Maternity Hospital; Department Of Medicine, Yildirim Beyazit University, Yeni Ziraat Mahallesi, 657. Street 5/9, Alt?nda?, Ankara, Turkey
- 2 Department Of Pediatric Nephrology, Dr Sami Ulus Childrens And Maternity Hospital, Ankara, Turkey
- 3 Department Of Ophthalmology, Dr Sami Ulus Childrens And Maternity Hospital, Ankara, Turkey
- 4 Department Of Pathology, Dr Sami Ulus Childrens And Maternity Hospital, Ankara, Turkey
*Corresponding Author:Sare Gülfem ÖZLÜ
Faculty Of Medicine, Department Of Pediatric Nephrology, Dr Sami Ulus Childrens And Maternity Hospital; Department Of Medicine, Yildirim Beyazit University, Yeni Ziraat Mahallesi, 657. Street 5/9, Alt?nda?, Ankara, Turkey
Received Date: Apr 22, 2016 Accepted Date: Jul 06, 2016 Published Date: Jul 20, 2016
The disease usually occurs between the ages of 3 and 15 years and there is a slight male predominance. It is a self-limiting disease in the majority of children and prognosis is excellent . Short-term morbidity and mortality is associated with gastrointestinal tract lesions and long-term prognosis is associated with severe renal involvement .
Uveitis can occur as manifestation of many rheumatological diseases most commonly Seronegative Spondyloarthritis (SSA), Behçet disease and juvenile idiopathic arthritis . The association of HSP and uveitis is very rare and to our knowledge there are only four patients reported (Table 1). Three of these patients are in adult age and one is 11 years old [3-5]. Here we report an 8-years-old boy who presented with uveitis and late onset renal involvement of HSP and also reviewed the literature.
|Features||Kaur ||Erer ||Yamabe ||Muqit ||Our patient|
|Age at onset of uveitis||11 years||39 years||64 years||42 years||13 years|
|Recurrence of uveitis||+||+||-||-||-|
|Treatment||Prednisolone||Prednisolone and cyclophosphamide||Conservative||Prednisolone||Prednisolone and azathioprine|
On laboratory investigation hemoglobin was 14.2g/dl, white blood cell count was 9900/mm3 platelet count was 417.000/mm3. C-Reactive Protein (CRP) was mildly elevated (15mg/dl, normal: 0-0,5); Erythrocyte Sedimentation Rate (ESR) was in normal limits. Renal function tests, urine microscopic analysis, liver function tests were normal. The Antinuclear Antibody (ANA) and rheumatoid factor were negative. Serology for Hepatitis B and C and Antineutrophil Cytoplasmic Antibody (ANCA) were negative. Throat swab was negative.
He was treated symptomatically by ibuprofen for arthralgia. Seven days later, the patient developed severe abdominal pain, nausea and vomiting. He had severe abdominal tenderness on physical examination. The laboratory tests showed elevated white blood cell count (18200/mm3) and CRP (29mg/dl) levels, and positive fecal occult blood test. There was no diagnostic finding on abdominal ultrasonography. Low dose steroids were given for a short duration for intestinal involvement. His clinical and laboratory findings improved gradually and he was discharged on the first week of hospitalization. Four weeks after the onset of the disease, asymptomatic mild proteinuria and microscopic hematuria were detected on urinalysis and continued for 8 weeks. Between 12 weeks -42 months during follow up, his physical examination and laboratory investigations were completely normal.
The patient was readmitted with pain and redness in his eyes 42 months after the onset of the disease. His physical examination was completely normal but ophthalmogical examination revealed bilateral anterior uveitis. There were no clinical signs and symptoms of renal involvement at this time. Fundus fluorescein angiography showed optic disc hyperfluorescence and capillary leakage. Visual acuity was intact in each eye. Differantial diagnosis for uveitis was investigated. There was no clinical and laboratory signs of infection and systemic inflammatory diseases. Acute phase reactants, serum complement levels, urinalysis, radiological examinations and echocardiography were all in normal limits. Antinuclear antibodies, anticytoplasmic antibodies and pathergy test were negative. Serum ACE levels were normal and he had no other sign and symptoms suggesting sarcoidosis. HSP was considered as a cause for uveitis, and azathioprine (2mg/kg/daily) and local steroids were started for treatment.
Six months later, he readmitted with rash and edema. Urinalysis showed hematuria and nephrotic range proteinuria. Hypoalbuminemia (3.3g/L) was detected. Glomerular filtration rate was 138ml/min/1.73m2and daily urinary protein loss 78mg/m2/h. ANA, anti ds-DNA antibodies, p- and c-ANCA were negative, and complement components normal. He was undergone renal biopsy because of heavy proteinuria and decrease in the serum albumin levels. Renal biopsy specimens showed diffuse mesengial proliferation and increase of the matrix (Figure 1), cellular (15/79 glomeruli) and fibrocellular crescents (1/79 glomeruli), global sclerosis (4/79 glomeruli), and fibrinoid necrosis and PMNL infiltration in a few glomeruli with deposition of mesangial IgA (+++/+++), C3 (++/+++) and fibrinogen (+/+++). These findings were defined as Grade III b HSP nephritis (diffuse mesangial proliferative glomerulonephritis) according to the classification of the International Study for Kidney Diseases in Children (ISKDC). We excluded tubulointerstitial nephritis and uveitis syndrome with renal biopsy findings. Ophthalmological examination revealed visual acuity exactly, anterior chamber scarring, peripheral vascular tortuosity in fundus (Clinical findings and treatment modalities are summarized in table 2). FFA was normal. He was administered oral prednisolone (2mg/kg/day) and ACE-?, and azathioprine was continued. At 3 months and 6 months follow up proteinuria improved and ocular findings were in normal limits.
Figure 1: Renal biopsy specimen showing mesengial proliferation.
|At admission||Only skin rash||Symptomatic|
|1st week||Skin rash + gastrointestinal involvement||Oral steroids for one week(1mg/kg/daily)|
|12th week||Normal physical examination||No treatment|
|42nd week||Bilateral anterior uveitis||Azathioprine (2mg/kg/daily)|
|48th week||Renal involvement (nephrotic syndrome)||
Although the patient reported in the study of Kaur et al.,  did not have any renal involvement, all of the three patients reported in the adult population had varying degrees of renal involvement [4,5]. Our patient had minimal lesions such as hematuria on microscopic evaluation and 1+ proteinuria on dipstick examination on the fourth week of the disease. The other important issue we want to emphasize is the late onset renal involvement of our patient. He was completely well during four years of follow up; he developed uveitis in the third year of the disease and after six months of uveitis in the fourth year of disease proteinuria and microalbuminuria occurred. In general nephropathy develops within a few days to 4 weeks after onset of the symptoms. Findings include microscopic or gross hematuria, proteinuria, nephritic or nephrotic syndrome and occasionally progressive renal failure . Retrospective studies detected that 20 to 54 percent of children with HSP had some degree of renal involvement . Although most patients with HSP nephritis have mild and self limiting disease about 20% of children with HSP nephritis will develop chronic kidney disease, with up to 2% progressing end stage renal disease [1,10]. Narchi et al.,  detected that renal manifestations occurred within four weeks in 84%, within six weeks in 91% and within six months in 97% of the patients and therefore they suggested that a six weeks of follow up will be sufficient. In a recent study of Tudorache E et al.,  the authors found that even mild HSP nephritis had been associated with long term proteinuria. Ronkainen et al.,  showed that 72% of patients in their cohort had renal symptoms after a mean follow-up of 19 years. In concordance with these studies our patient developed glomerulonephritis in the fourth year of the disease and in spite of immunosuppressive treatment.
In conclusion we would like to remind the importance of long term follow up in HSP even in the absence of significant renal findings at the beginning of the disease. Although it is not a part of routine examination in HSP; because of the possibility of uveitis we suggest an ophtalmological examination in these patients. Besides the paucity of the literature on this issue we speculate that uveitis may be more common in children with HSP nephritis.
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Citation:ÖZLÜ SG, YAZILITA? F, AYDO? O, BÜLBÜL M, YILDIZ C, et al. (2016) Uveitis and Late Onset Nephritis Associated With Henoch Schonlein Purpura: A Case Report. J Nephrol Renal Ther 2: 007
Copyright: © 2016 Sare Gülfem ÖZLÜ, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.