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A Case Report on Henoch-Schonlein Purpura



Prince Shah1, Mohit Buddhadev2, S P Srinivas Nayak2*, Gunosindhu Chakraborthy3

1PharmD, Parul Institute of Pharmacy and Research, Parul University, Vadodara, Gujarat, India

2Assistant Professor, Department of Pharmacy Practice, Parul Institute of Pharmacy and Research, Parul University, Vadodara, Gujarat, India

3Professor & Pincipal, Parul Institute of Pharmacy and Research, Parul University, Vadodara, Gujarat, India

 

*Corresponding author: S P Srinivas Nayak, Assistant Professor, Department of Pharmacy Practice, Parul Institute of Pharmacy and Research, Parul University, Vadodara, Gujarat, India. Email: [email protected]  

 

Received Date: 08 October, 2021; Accepted Date: 16 October, 2021; Published Date: 12 October, 2021

Henoch-Schonlein purpura (HSP) also known as IgA vasculitis is a disorder that causes the small blood vessels in your skin, joints, intestines, and kidneys to become inflamed and bleed. The most striking feature of this form of vasculitis is a purplish rash, typically on the lower legs and buttocks. Henoch-Schonlein purpura can also cause abdominal pain. This case report describes a 13 year old female patient presented with complains of Rashes over both upper and lower limb, black stool, blood in urine since last 10 days, swelling over both limbs. Other complaints included blood in vomit. diagnosed with Henoch Schonlein Purpura with Grade III A nephritis with leukocytoclastic vasculitic changes. Patient was kept on antibiotics, for over a period of 2 weeks to prevent any hospital acquired infection. Also Steroids is added. Early recognition of multiorgan involvement, especially early age group, and appropriate intervention can mitigate the disease and limit organ damage.

 

Keywords: Henoch-Schonlein purpura; Case report; Vasculitis; Multiorgan disease

Introduction

Henoch-Schonlein Purpura (HSP) is a self-limited, systemic, nongranulomatous, autoimmune complex, small vessel vasculitis, with multiorgan involvement. Its etiology is unclear but is associated with infections (bacterial, viral, parasitic), medications, vaccination, tumors (non-small cell lung cancer, prostate cancer, and hematological malignancies), alpha-1-antitrypsin deficiency, and Familial Mediteranean Fever [1].  HSP is the most common cutaneous vasculitis in children comprising up to 90% of cases [1-3]. HSPN is caused by the glomerular deposition of immunoglobulin A1 (IgA1) containing immune complexes in the mesangium, the subepithelial and the subendothelial space. Formation of the IgA1 immune complex is thought to be the consequence of aberrantly glycosylated IgA1 molecules secreted into the circulation and their subsequent recognition by IgG specific for galactose-deficient IgA1 [4]. Mesangial proliferation and renal damage are triggered by the deposited immune complexes, which likely require activation of the complement system. Whereas other organ manifestations of HSP are mostly benign and self-limiting, HSPN might lead to chronic renal disease and end stage renal failure, thereby justifying immunosuppressive treatment. The patient quite requisites all the feature od classic HSPN [5]. HSP is characterized by a classic tetrad of nonthrombocytopenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement, and rarely, other systems (lungs, central nervous system, genitourinary tract) [2,6]. Cutaneous involvement is the most common presentation, although patients may present with involvement of other organ systems. Although HSP is uncommon in the adolescent age group, non-thrombocytopenic palpable purpura with multiorgan involvement (gastrointestinal, kidney and joints) should make one consider the diagnosis. Prompt diagnosis and multidisciplinary intervention can lead to appropriate management and mitigate potential complications, as illustrated in this case. Treatment of HSP reflects its self-limiting nature in 94% of children and 89% of adults. Symptomatic treatment will be sufficient for symptoms such as rash and arthritis. Acetaminophen and nonsteroidal anti-inflammatory drugs can be used [4]. Aspirin should be avoided in children. Oral steroids are indicated in patients with severe rash, edema, severe colicky abdominal pain (without nausea, vomiting), renal, scrotal, and testicular involvement. Usually prednisone or methylprednisolone can be started at 1 to 2 mg/kg per day for one to two weeks, tapering down to 0.5 mg/kg/day over the next week and then 0.5 mg/kg every other day for one more week. Intravenous (IV) steroids can be administered if the patient does not tolerate oral steroids. Early steroid therapy decreases gastrointestinal symptoms within 2 days compared to 12.3 days in patients without steroids and may decrease HSP or gastrointestinal recurrence and reduce renal progression. Steroids may prevent major complications such as gastrointestinal bleeding or intussusception [7-10]. High-dose IV pulse steroids are indicated in patients with nephrotic range proteinuria and mesenteric vasculitis. These pulse doses can range from 500 mg to 1 gm with various protocols leading to complete remission of nephritis in a few studies [8]. All patients with severe renal involvement should be referred to the nephrologist and a renal biopsy is recommended.

 

Case Presentation

A 13 year old female patient arrived at SSG Hospital, Vadodara along her parents with complains of Rashes over both upper limb and lower limb, black stool, blood in urine since last 10 days, swelling over both limbs. Other complaints included blood in vomit.  Patient had a history of injury in right foot, this was the incident that made patient to visit the hospital. Her underlying condition was not diagnosed yet.

 

Physical Examination

Upon physical examination, patient had mild pallor. On General examination the patient was fair, cooperative and coherent; CNS: Conscious and oriented; CVS: S1, S2 (positive), murmurs not noted; RS: BLAE (+); GIT: soft, non-distended.

 

Vitals

Respiratory rate (RR): 28 breaths/minute; Pulse rate (PR): 114 beats/minute; Temperature: 98.2°F; Oxygen saturation (SpO2): 97%.

 

Family History and History of Present Illness

The patient was 4th child from a total of 7 children. No other brother or sisters had any sort of congenital deformities or any sort of inflammatory disorder. Mother was perfectly normal and the delivery of patient was vaginal delivery. There were no complications faced during pregnancy. No history of similar illness in any other first degree or second degree relative.

 

Laboratory Findings

Diagnostic Tests

  • Factor XIII Assay :- Deficiency Detected
  • IgGab to DsDNA :- 1.57 (06/08/2021)
  • Amylase :- 23
  • Lipase :- 28
  • Gastric Biopsy :- The Section shows changes of Leukocytoclastic Vasculitis
  • IgA FITC :- +ve +2
  • Complement c1q FITC :- +ve +2
  • ANA :- 1:100 --- Negative
  • ANC :- 1:100 ----- Negative
  • Complement 3 level :- 1.070 (0.90-1.80gm/L)
  • Complement 4 level :- 29.16 (10-40mg/dl)
  • Glomerulo Basement Membrane :- 5 (RU)               (<20 RU/ml)

 

Final Diagnosis

Correlating subjective and objective data along with past history, final diagnosis was made to be Henoch Schonlein Purpura with Grade III A nephritis with leukocytoclastic vasculitic changes.

 

Plan of Analysis

Discussion

A 13 year old patient presented to the hospital with the symptoms of Henoch Schonlein Purpura. The patient was previously undiagnosed and was in a sway between hospitals and hence the severity of the disease increased. Since the patient showed up with symptoms such as Rashes over both upper limb and lower limb, black stool, blood in urine since last 10 days, swelling over both limbs. Other complaints included blood in vomit. All together indicated vasculitis but to rule out the specific type diagnostic tests were done and further led to HSPN. The main reason of concern here was the leukocytoclastic changes were too much evident in the gastric biopsy and Blood in urine. Urinary blood was a symptom of vasculitis, but to lower it several medication had to be given or avoided. Since renal failure is associated with many narrow therapeutic drugs.

 

Conclusion

HSPN at this age is quite treatable but with very less documentation available for the treatment. The main concern behind this was recurrence and remission, anything is possible and we have very less documents available for our referrals. Patient had lost too much that needed to monitored throughout life and periodic checkups at certain time intervals is really necessary. Use of colistin and levofloxacin isn’t justified here since the renal function is already affected due to vascular effects. Levofloxacin itself is known to cause HSPN and rhabdomylsis if used chronically. Overall, the patient recovered successfully. Early recognition of multiorgan involvement and misuse of certain drugs can prevent further consequences.

 

No Figures

Table 1: Laboratory findings.

Lab Test

16/8

17/8

20/8

21/8

 

Hb (12-17.5)

8.4

8.4

8.6

8.3

 

WBC

1000

1000

1800

3000

 

AST

-

28

-

-

 

ALT

-

18

-

-

 

Bilirubin Total

-

0.7

-

-

 

Bilirubin Direct

-

0.4

-

-

 

Bilirubin Indirect

-

0.3

-

-

 

Total Protein

-

5.2

-

-

 

Albumin

-

2.2

-

-

-

Serum Electrolytes

-

-

-

-

-

CRP

-

17

-

-

-

Na+

-

128

136

-

-

K+

-

2.9

4

-

-

S. Creatinine

-

0.62

0.85

1.17

-

BUN

-

43

27

29

28

Ferritin

822

-

--

-

 

TG

233

-

-

--

-

Urine Protein

442.53

-

-

-

-

Urine Creatinine

110.45

-

-

-

-

PT

18.3

-

-

-

-

INR

1.3

-

-

-

-

Control

13.5

-

-

-

-

aPTT

31.5

-

-

-

-

NT-ProB-Type Natriuretic Peptide H (0-145)

275.3

-

-

-

-

 

Table 2: Drug Management.

DRUG

INDICATION

DOSE

ROUTE

FREQUENCY

Inj MEROPENEM

PROPHYLAXIS (Antibiotic)

10mg/kg/d

IV

TDS

Inj Vancomycin

PROPHYLAXIS (Antibiotic)

15mg/kg/d =375mg

IV

OD

Inj PCM

PAIN

10mg/kg/d = 350mg

IV

SOS

Inj Emset

Vomiting

0.15mg/kg/d = 5mg/kg/d

IV

SOS

IVIG Cryoppt

IgA Replacement

1g/kg/d = 35gm @ 1ml/min

IV

OD

Clotrimazole Lozenges

PROPHYLAXIS (Antibiotic)

10mg

PO

1 BD

Colistin + Levofloxacin

PROPHYLAXIS (Antibiotic)

 

 

 

Inj GMCSF (Filgrastim)

Lowered WBC

@2mg/kg/d in 2 doses = 0.4ml

IV

OD

Inj Methyl Prednisolone

Reduce inflammation

@1.6mg/kg/d

IV

OD

Vit C

Immunity Booster

-

IV

OD

Pantoprazole

PPI

-

IV

 

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Citation: Shah P, Buddhadev M, Srinivas Nayak SP, Chakraborthy G (2021) A Case Report on Henoch-Schonlein Purpura. Open J Case Rep 2: 151.