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LETTER TO EDITOR  
Year : 2021  |  Volume : 64  |  Issue : 5  |  Page : 180-181
Acute pancreatitis as a presenting feature in a child with systemic polyarteritis nodosa


Department of Pediatrics, Bharati Vidyapeeth University Medical College Hospital and Research Centre, Pune, Maharashtra, India

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Date of Submission01-Jul-2020
Date of Decision31-Jul-2020
Date of Acceptance28-Oct-2020
Date of Web Publication7-Jun-2021
 

How to cite this article:
Kapur SV, Oswal JS. Acute pancreatitis as a presenting feature in a child with systemic polyarteritis nodosa. Indian J Pathol Microbiol 2021;64:180-1

How to cite this URL:
Kapur SV, Oswal JS. Acute pancreatitis as a presenting feature in a child with systemic polyarteritis nodosa. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 Jun 13];64:180-1. Available from: https://www.ijpmonline.org/text.asp?2021/64/5/180/317927




Dear Editor,

Sytemic Polyarteritis Nodosa (PAN) is a rare multi-systemic necrotizing, focal segmental vasculitis that affects predominantly medium-sized vessels sparing the lungs. Identification of other forms of vasculitis as distinct entities has significantly narrowed down the spectrum of PAN. The diagnosis of PAN should be confirmed by biopsy whenever possible. There is very limited data with regards to gastrointestinal involvement (GI) in PAN in children.

A 15-year-old male child presented with severe abdominal pain with multiple episodes of vomiting of two week duration. There was no history of fever, loose stools, weight loss, myalgia, joint pains, ulcers, or peripheral neuropathy. On examination, he had normal blood pressure, nodular skin rash over his legs and his abdomen was diffusely tender. Investigations revealed anemia (haemoglobin- 8.1 gm/dl), increased WBC counts (WBC count- 18,900/mm3 neutrophils = 68, lymphocytes = 32) and thrombocytosis (platelet count- 4.1 lakhs/mm3) with raised acute phase reactants (Erythrocyte sedimentation rate- 64 mm/hr and C-reactive protein -16 mg/L). Liver function, urine routine, renal function, lipid profile, coagulation profile, stool for occult blood, ophthalmology examination, 2D-Echocardiography, and X-ray chest were normal with low albumin (2.5 g/dl), low serum calcium (7.6 mg/dl), elevated amylase (2300 U/L) and lipase levels (3100 U/L). Abdominal CT scan revealed a diffusely enlarged pancreas with heterogenous parenchyma and mild peripancreatic inflammation consistent with acute pancreatitis. The viral serology markers (HIV, HBsAg, HCV) were negative. Skin biopsy revealed leukocytoclastic vasculitis of medium-sized artery with massive cellular infiltrate in the deeper dermis/subcutis consisting mostly of polymorphonuclear cells suggestive of PAN [Figure 1]a. Antinuclear antibody (ANA), anti-double stranded DNA (anti-dsDNA), Anti-neutrophil cytoplasm antibodies (ANCA), C3, C4, and antiphospholipid antibodies were negative. CT angiogram of abdomen revealed extensive constriction, dilatation and aneurysms affecting coeliac trunk and pancreaticoduodenal arteries [Figure 1]b. CT chest showed no features of pneumonia or granulomatous disease. Based on the clinical scenario (acute pancreatitis with skin rash), laboratory investigations (raised WBC, platelets, and acute phase reactants), skin biopsy finding of leukocytoclastic vasculitis and abnormal angiography, a diagnosis of acute pancreatitis secondary to PAN was made. The patient was treated with pulse steroids and monthly cyclophosphamide infusions. He has significant clinical improvement with normalization of hemogram and acute phase reactants at 4 months of follow up.
Figure 1: (a) Skin biopsy showing leukocytoclastic vasculitis of medium sized artery with massive cellular infiltrate in the deeper dermis/subcutis consisting mostly of polymorphonuclear cells (H and E, ×100). (b) CT Angiogram suggestive of multiple constriction, dilatation and aneurysms formation of celiac trunk and pancreaticoduodenal arteries

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PAN requires histopathology evidence of necrotising vasculitis or angiographic abnormalities of medium/small-sized arteries plus one of five criteria: (1) skin involvement, (2) myalgia, (3) hypertension, (4) peripheral neuropathy, and (5) renal involvement.[1] Our patient presented with acute pancreatitis and only had a skin rash with no other signs which led to a diagnostic dilemma. Skin biopsy confirmed the diagnosis of PAN which was later supported by angiography. In addition, he had anemia, leukocytosis, thrombocytosis, and elevated acute phase reactants as would be expected normally in a case of vasculitis. Other potential site for biopsy includes sural nerve, muscle, or testes.

GI involvement, a major cause of morbidity and mortality in systemic PAN occurs in 14% to 65% of patients.[2] GI manifestation includes pancreatitis, pancreatic infarction, bowel infarction and perforation, cholecystitis, or hepatic infarction.[3] However, it is to be noted this data in mainly taken from adult patients. To the best our knowledge, there is no Indian published report with regards to association of acute pancreatitis with PAN in children. Genetic workup for Adenosine deaminase 2 deficiency (DADA2) as a cause of early onset PAN was not done in this child due to financial constraints.[4]

We illustrate this rare case to highlight acute pancreatitis as a presenting feature in a child with systemic PAN, the diagnosis of which was based on the histopathological study. Abdominal pain may be the first presenting symptom of vasculitis, and such cases require careful clinical examination and work up including a tissue biopsy. Prompt treatment with corticosteroids and immunosuppressive agents may impact disease prognosis if the diagnosis of PAN can be confirmed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for henoch-schönlein purpura, childhood polyarteritis nodosa, childhood wegener granulomatosis and childhood takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 2010;69:798-806.  Back to cited text no. 1
    
2.
Levine SM, Hellmann DB, Stone JH. Gastrointestinal involvement in polyarteritis nodosa (1986–2000): Presentation and outcomes in 24 patients. Am J Med 2002;112:386-91.  Back to cited text no. 2
    
3.
Thorne JC, Bookman AA, Stevens H. A case of polyarteritis presenting as abrupt onset of pancreatic insufficiency. J Rheumatol 1980;7:583-6.  Back to cited text no. 3
    
4.
Caorsi R, Penco F, Grossi A, Insalaco A, Omenetti A, Alessio M, et al. ADA2 deficiency (DADA2) as an unrecognised cause of early onset polyarteritis nodosa and stroke: A multicentre national study. Ann Rheum Dis 2017;76:1648-56.  Back to cited text no. 4
    

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Correspondence Address:
Sunil V Kapur
Department of Pediatrics, Bharati Vidyapeeth University Medical College Hospital and Research Centre, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_804_20

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