Speaker Name: Dr. Sandeep Kumar Reddy
Category: (Speaker/Oral Presentation)
Biography: Madras medical college TNMGRU Chennai
Global Journal of Gastroenterology & Hepatology Research [GJGHR]
ABSTRACT:
Henoch–Schönlein purpura (HSP), also known as IgA vasculitis, is a systemic vasculitis which is the most common vasculitis in children.
The incidence in adults varies from 3.4 to 14.3 cases per million.
The classic triad of symptoms include purpuric rash, arthritis and abdominal pain.
KEY WORDS: small vessel vasculitis;rash;abdominal pain.
We present a case of a 45 year-old female with HSP who presented with recurrent episodes of abdominal pain, followed by classical symptoms with an identified post-infectious etiology.
A 45 year old female with no previous medical history presented with a florid purpuric rash affecting distal extremities, abdominal pain and a monoarthritis of her wrist.
Two weeks prior she had attended casualty with a flu-like illness and a swollen tender right ankle, but subsequently discharged without workup.
Over the course of next week she began to develop a purpuric rash on her distal extremities spreading to buttocks .On presentation she developed central crampy abdominal pain, increasing in severity over the previous 2 days with no relief with simple analgesics.
On initial examination her right wrist was swollen and tender on palpation. She remained afebrile with temperature of 37celsius and B.P 128/80 mmHg, heart rate of 78 bpm and oxygen saturation of 98% on room air.
No other obvious infective sources were identified on initial examination with no evidence of meningism.
Inflammatory markers were raised with a white cell count of 12.8 × 10 9 /L and C-reactive protein of 48.6 mg/L. Coagulation profile was normal with platelet count 442 × 10 9 /L. ESR was elevated at 48 mm/h.
Urinalysis revealed a trace of protein.
Diagnosis and initial management
A working diagnosis of Henoch–Schönlein purpura (HSP) was made and she was started on oral prednisolone along with protein pump inhibitor (PPI). Further investigations sent included immunoglobulin (Ig) profile, complement level and albumin creatinine ratio.
Anti-streptolysin O titre was sent as the patient had sore throat two weeks before.On day two of admission the purpuric rash was less pronounced, and serum Ig levels revealed an elevated IgA of 5.40 g/L (0.8–2.8 g/L)
CECT abdomen-Diffuse circumferential regular wall thickening in antro-pyloric region p/o Gastritis.
Ogd showed-Diffusely inflammed stomach with erosions.multiple tiny ulcers in duodenum with nodularity.Biopsy-Active duodenitis.
Skin biopsy showed :flaky inflammatory infiltrate around blood vessels.Fibrinoid degeneration of vessel wall seen.No thrombosis inside tubular blood vessels.
Inflammatory infiltrate consists of mononuclear cells, lymphocytes around hair follicles.Impression: Features suggestive of cutaneous small vessel vasculitis.
Over the next 24 hours she began to experience abdominal pain and vomiting with increasing severity requiring opioid analgesia. On examination there was an improvement in purpuric rash, and the abdomen was soft with no guarding or rebound tenderness and bowel sounds were heard. Arterial blood gas revealed a metabolic alkalosis with pH 7.53, bicarbonate (HCO 3 ) standard 29.7 mmol/L and lactate 2.2 mmol/L. Abdominal film showed non-specific gas pattern with no obvious perforation or obstruction. Full blood picture showed a rise in white cell count to 21.4 ×10 9 /L with a prominent neutrophilia.
In view of gastrointestinal (GI) haemorrhage and bowel wall oedema associated with the condition, treatment was changed to course of intravenous (IV) methylprednisolone with IV PPI cover.The patient’s abdominal pain and vomiting subsequently improved with a combination treatment of 3 days IV methylprednisolone, IV fluids and antiemetics. Five days after initial presentation, purpuric rash had resolved along with right wrist monoarthritis. Prior to discharge anti-streptolysin O titre elevated at 400 IU/L and a diagnosis of HSP precipitated by streptococci infection was made. She was subsequently discharged on a slow tapering dose of oral prednisolone, and reviewed in opd 8 weeks post-admission with a complete resolution of symptoms.
Discussion:
HSP is a small vessel vasculitis in which immune complexes of IgA and complement component 3 (c3) are deposited on capillaries, venules and arterioles. In adults, HSP has been observed to have a higher frequency of systemic involvement and the outcome in adults was found to be similar to children with complete recovery from the disease in the majority of cases. The typical symptoms include purpuric rash appearing on legs and buttocks however the rash may also be seen on the arms, face and trunk. Joint arthritis involves ankles, knees and elbows and is non-erosive hence does not cause permanent deformity.40% of patients will have renal involvement, mainly in the form of haematuria which is predominantly microscopic in nature.
Proteinuria occurs in more than half of patients involved, some of which is severe enough to cause nephrotic syndrome. Approximately 1% of all HSP patients go on to develop chronic kidney disease, and adults are more likely to do so than children. Clinically, since there are no disease-specific laboratory abnormalities, HSP is currently diagnosed based on symptoms,signs and histopathology.
To date, several diagnostic criteria have been proposed with the most recent gold standard EULAR/PRINTO/PRES criteria 2010.GI symptoms occur in about one half of HSP patients and range from mild symptoms to more significant conditions such as intussusception ,GI haemorrhage, bowel ischaemia and perforation.In a study involving HSP in the adult population, 24.1% of patients developed GI symptoms prior to the cutaneous rash. Abdominal pain is typically colicky in nature, poorly localized, and can be accompanied with vomiting and bloody diarrhoea.
Treatment remains primarily supportive in most cases and hospitalisation should be considered for patients with severe abdominal pain, significant GI bleeding or marked renal insufficiency.
Supportive measures include adequate hydration and monitoring for abdominal and renal sequelae. Any unnecessary drugs should be discontinued if a drug-related aetiology is being considered.
Drugs associated with development of HSP include antibiotics such as vancomycin, cefuroxime, ACE inhibitors and diclofenac.More than 75% of patients report prior history of upper respiratory tract or pharyngeal infection. The development of HSP has been associated with multiple bacterial and viral infectious agents.
Examples include group A streptococcal infection, mycoplasma, hepatitis B and herpes simplex virus.
Due to the high spontaneous recovery rate most patients do not require specific therapy and steroids are generally avoided.
Steroids may shorten the duration of abdominal and joint pain but must be weighed up against potential adverse effects. Abdominal symptoms in HSP are caused by haemorrhage and oedema within the bowel wall and mesentery. The pathophysiology of steroid use for GI may be attributed to the reduction edema.
References:
1)Blanco R , Martínez-Taboada VM , Rodríguez-Valverde V , GarcíaFuentes M , González-Gay MA . Henoch–Schönlein purpura in adulthood and childhood: two different expressions of the same syndrome . Arthritis and Rheumatism 1997
2)Srestha S , Sumingan N , Tan J et al . Henoch–Schönlein purpura with nephritis in adults: adverse prognostic indicators in a UK population . QJM 2006 ; 99 : 253 – 65 . Ozen S , Pistorio A , Iusan SM et al . EULAR/PRINTO/PRES criteria for Henoch–Schönlein purpura
3)Zhang Y , Huang X . Gastrointestinal involvement in HenochSchönlein purpura . Scand J Gastroenterol 2008 ; 43 : 1038 – 43 .
4)Rigante D , Castellazzi L , Bosco A , Esposito S . Is there a crossroad between infections, genetics, and Henoch–Schönlein purpura? Autoimmun Rev 2013 ; 12 : 1016 – 21 .
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