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 . 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 . 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 . 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 . 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 . All patients with severe renal involvement should be referred to the nephrologist and a renal biopsy is recommended.
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.
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.
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.
- 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)
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
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.
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.