Kembara Xtra - Medicine -Henoch-Schönlein Purpura

​Kembara Xtra - Medicine -Henoch-Schönlein Purpura

More commonly known as immunoglobulin A vasculitis (IgAV)
Henoch-Schönlein purpura (HSP) is a nonthrombocytopenic, primarily IgA-mediated, small vessel vasculitis that affects multiple organ systems and can affect both children and adults. HSP is frequently self-limited, with the majority of morbidity and mortality being attributed to long-term renal damage.


Prevention
​Incidence
90% of HSP patients are under the age of 10; however, cases have been observed in patients as young as 6 months old and as old as 75. The annual incidence ranges from 3 to 27 per 100,000 children and 0.8 to 1.8 per 100,000 adults.
Gender: The ratio of men to women ranges from 1.2:1 to 1.8:1.
The majority of Caucasians and Asians experience it, but African Americans experience it less frequently. 
Prevalence
Prevalence yearly: 10 to 22/100,000 people
All year round; most frequent in the fall 

Pathophysiology and Etiology 

IgA1 immune complexes subsequently activate the complement system, causing the generation of inflammatory cytokines and chemokines. This is an autoimmune condition in which IgA production is elevated in response to a trigger or many triggers.
IgA-containing immune complex deposition causes inflammation of the tiny blood vessels, which in turn causes fibrosis and necrosis in the skin, intestinal mucosa, joints, and kidneys.
● Although no single etiologic agent has been found, there are a few associations (mentioned below), and a multihit model leading to HSP-associated glomerulonephropathy is a widely accepted idea.
– Prevalence in the fall and a typical upper respiratory infection (URI) prodrome point to infections.
Associated pathogens include (but are not limited to) group A Streptococcus (may be present in up to 30% of HSP-associated nephritis), parvovirus B19, Bartonella henselae, Helicobacter pylori, Haemophilus parainfluenzae, coxsackievirus, adenovirus, hepatitis A and B viruses, Mycoplasma, Epstein-Barr virus, herpes simplex, varicella, Campylobacter, methicillin-resistant Staphylococcus aureus.
– Acetaminophen, angiotensin-converting enzyme inhibitors (ACEI), angiotensin II receptor antagonists (ARB), certain antibiotics, especially clarithromycin quinolones, etanercept, codeine, and nonsteroidal antiinflammatory medications are the pharmaceuticals most frequently associated with people with HSP.
– Rarely have vaccinations been linked to HSP; nevertheless, there have been occasional instances involving the administration of the MMR (measles, mumps, rubella), pneumococcal, meningococcal, influenza, and hepatitis B vaccines.
Genetics
Linked to SNPs in the genes for HLA-A2, HLA-A11, HLA-B35, HLA-DRB1, renin-angiotensin, and nitric oxide synthase, as well as familial Mediterranean fever.

Accompanying Conditions 

Malignancy: Although uncommon, this condition is most frequently associated with solid tumors, such as lymphoma, prostate cancer, non-small cell lung cancer, and multiple myeloma.
Studies point to a potential link between immune-related illnesses (inflammatory bowel disease, medication allergies, and food allergies) and H. pylori infection.

Diagnoses include at least one of the following and palpable purpura/petechiae without thrombocytopenia: 
Diffuse abdominal discomfort, IgA deposition in the majority of biopsies, arthritis, arthralgia, renal involvement (hematuria or proteinuria), and direct immunofluorescence demonstrating IgA deposition


Rash (the most common presenting symptom): purpuric, palpable; predominant distribution often symmetric on dependent areas (lower extremities and buttocks); may spread elsewhere; typical duration 3 to 10 days; no clear temporal association with other symptoms. Presenting History: Exposure to potential trigger, including recent infection (particularly URI) or offending drug.
Gastrointestinal (GI), which accounts for 50–75% of cases. - Vomiting/nausea
- Colicky, diffuse abdominal pain that may be continuous or fleeting - Melena/Hematochezia
Polyarthritis, which affects roughly 75% of cases - The knees and ankles are frequently involved symmetrically Renal (approximately 20–55% of cases) - Gross hematuria (more common in adults than in kids)
Oliguria or anuria, fatigue, low-grade fever, and rare symptoms include hemoptysis, periorbital or scrotal enlargement, headache, convulsions, neuropathy, and behavioral changes

clinical assessment 
Rash is the predominant presenting symptom in 96% of cases. It may begin as urticaria and progress to a palpable, non-blanching purpura with or without petechiae, ecchymoses, and bullae. - Distribution is often symmetrical and most frequently affects the lower extremities, though it can also affect the face and trunk.
- Rectal examination is advised to check for hematochezia in cases where there is evidence of GI bleeding (28% of cases), which causes abdominal soreness (66% of cases, 12% of cases).
Joint soreness (64 percent of cases, 15 percent of cases): Mainly affects the knees or ankles; may be accompanied by warmth and restricted range of motion; effusion is less frequently present; erythema is absent.
– the majority are nondeforming, transitory, and nonmigratory

Orchitis (5%): - Shows up as scrotal discomfort and swelling, with possible torsion.
Renal disease: Hypertension may be present (1% primary presenting symptom).
Rarely, patients exhibit central nervous system (CNS) or pulmonary involvement, which may show as symptoms of diffuse interstitial pneumonia or cerebral hemorrhage, respectively.
Adults have lower extremities edema more frequently than kids do.


Meningococcemia, Rocky Mountain spotted fever, bacterial endocarditis, rheumatic fever, Epstein-Barr virus, and sepsis are among the infections with different differential diagnoses.

Vasculitides include Systemic lupus erythematosus, Polyarteritis nodosa, Granulomatosis with Polyangiitis, Microscopic Polyangiitis, and Kawasaki illness.
- Cryoglobulinemia-associated vasculitis - Urticarial vasculitis - Other:
- IBD - Acute glomerulonephritis due to streptococcal infection
- Juvenile idiopathic arthritis, mixed connective tissue disease, juvenile dermatomyositis, idiopathic thrombocytopenic purpura, thrombotic thrombocytopenic purpura, leukemia, and lymphoma
- Acute surgical abdomen - Hereditary hemorrhagic telangiectasia - Abuse of children

Laboratory Results 

There isn't a single lab test that can definitively rule out other diseases or determine the extent of renal involvement in HSP. 
Initial examinations (lab, imaging)

The following tests are typically used as the first ones for HSP:
Leukocytosis and thrombocytosis may be present on a complete blood count.
Eosinophilia is widespread. Thrombocytopenia suggests an additional purpura etiology.
- Depending on whether GI bleeding occurs, hemoglobin varies.
Basic serum chemistry panel: Elevated creatinine or electrolyte imbalances point to renal failure.
Urinalysis: Red cell casts, proteinuria, and gross or microscopic hematuria all point to renal failure.
Partial thromboplastin time and prothrombin time/international normalized ratio are both normal in HSP. Alternate causes of purpura may be revealed by abnormal coagulation testing.
Imaging is not a component of the standard treatment for HSP, although it may be done to rule out alternative causes or to assess potential consequences, especially when the GI or the kidneys are involved. The following are some initial imaging modalities to take into account: - Abdominal radiography, either with or without a barium enema, to check for abdominal air that could indicate a bowel perforation - Abdominal ultrasound: sensitive for detecting intramural hemorrhage in HSP, as well as showing thicker gut wall, decreased peristalsis, and intussusception.
In cases of renal insufficiency, renal ultrasonography assesses for hydronephrosis. Tests in the Future & Special Considerations
Acute phase reactants (erythrocyte sedimentation rate (ESR)/C-reactive protein): - To rule out sepsis/bacteremia when diagnosis is ambiguous Blood culture: - To confirm HSP - - Expect a slight increase

Complement levels are often normal or slightly lowered. IgA levels are frequently raised but nonspecific and insensitive. Antinuclear and antineutrophil cytoplasmic antibodies are negative but aid in reducing the differential diagnosis.

Stool guaiac if suspected GI hemorrhage; Antistreptolysin-O titer: - Assesses for prior streptococcal infection; CT angiography may be required to pinpoint the site of bleeding in patients with GI hemorrhage.

Other/Diagnostic Procedures
If the diagnosis is unclear or if a urinalysis reveals proteinuria in the nephrotic range, get a renal biopsy. A biopsy might reveal crescentic glomerulonephritis, mesangial IgA deposition, or mesangial proliferation.

IgA accumulation in the dermis on immunofluorescence in a purpura skin biopsy
Due to the clinical overlap between HSP and inflammatory bowel disease, endoscopy may be examined in cases of GI hemorrhage. Barium enema may be helpful in some cases of intussusception, while surgical correction is usually required.

Management 

Purpura may be curbed with rest and elevation of the affected areas.
Nutrition and hydration are helpful during treatment.

Medication 
HSP is typically self-limited and best managed with supportive treatment when there is no renal impairment or consequence since it resolves in 94% of children and 89% of adults.
NSAIDs are beneficial for treating joint pain symptoms. In cases of renal involvement, caution is suggested; acetaminophen may be used instead.
In patients with severe joint and stomach pain as well as those with glomerulonephritis with extensive renal involvement, steroids are helpful early in the course of the disease. Prednisone used orally (1 to 2 mg/kg) may lessen joint pain intensity and duration. This could help with GI bleeding prevention and surgical abdominal reasons such intussusception.
- The use of steroids in the treatment of severe and/or bullous purpura is beneficial.
– When given early in the course of the illness, steroids can effectively cure crescentic nephritis in its acute stage and help these people avoid developing chronic renal impairment.
- After a year, early steroids have no impact on renal involvement development or prevention.
Cyclosporine [Sandimmune] and mycophenolate [Cellcept] immunosuppressive therapy may be advantageous for patients who have steroid-resistant illness and show signs of significant renal damage. Numerous small-scale case studies and case reports have demonstrated the advantages for these patients. Small trials have all detailed the use of high-dose IV pulse steroids, cyclophosphamide, rituximab, mycophenolate, and plasmapheresis.
The timing of when to utilize these medications conclusively is still up for debate and needs more study.
Patients with HSP and persistent proteinuria may find benefit from ACEI or ARB.


Referral 

If you have ever had proteinuria in the nephrotic range or have had proteinuria >100 mg/mmol for three months after your diagnosis, you should think about having a kidney biopsy.
If the diagnosis is ambiguous, the patient might be sent to a dermatologist for a skin biopsy. 

Admission Initial stabilization Admission requirements - Insufficient oral intake - Renal insufficiency - Severe GI bleeding - Altered mental condition - Arthritis-related mobility restrictions - Hypertension - Nephrotic syndrome
IV fluids: It's important to keep hydrated.

Patient Follow-Up Monitoring
During the acute illness, patients should be seen every week.
An examination should include a history, physical (including a blood pressure reading), and a urine test.
All patients should be monitored with BP and urinalysis at least monthly for a minimum of 6 months because 100% of individuals who acquire renal involvement do so within 6 months of HSP diagnosis.
Pregnant women with a history of HSP should have their proteinuria and blood pressure checked.
When treating patients with adult-onset HSP, take occult malignancy into consideration.


Long-term prognosis is greatly influenced by the prevalence and severity of nephritis. In 94% of children and 89% of adults, HSP is self-limited.
Most HSP cases resolve 4 weeks after diagnosis.
Within six months of diagnosis, the recurrence rate is 33%.
Age above 8 years, fever at presentation, purpura above the waist, higher ESR or IgA concentration, and increasing severity of renal histology grade are all factors that are linked to a worse prognosis.
Compared to adults who had any renal involvement, 50% of children with nephritic and nephrotic syndrome have chronic renal illness. There is a 5% chance of long-term renal failure.
Old age, HTN, high blood creatinine, nephrotic and mixed nephritic/nephrotic syndrome at disease initiation are risk factors that can lead to renal failure.

Complications include cerebral hemorrhage, seizures, intestinal infarction, perforation, obstruction, nephrotic/nephritic syndrome, hemorrhagic cystitis, ureteral obstruction, gastrointestinal (GI) hemorrhage, intussusception, and alveolar hemorrhage.
Testicular torsion, myocarditis, anterior uveitis and orchitis, 

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