Schonlein henochova purpura

CMAJ. Upper respiratory tract infections are the most common; however, patients may also present with an antecedent gastrointestinal or pharyngeal infection.

Group A Streptococcus has been found in cultures of greater than 30% of patients with IgAV with nephritis.[5][6] 

Other causative agents include.[5]

  • Coxsackie virus

  • Hepatitis A, hepatitis B (and hepatitis vaccines)

  • Mycoplasma

  • Parvovirus B19

  • Infectious mononucleosis

  • Subacute bacterial endocarditis

  • Helicobacter pylori

  • Yersinia, Shigella

  • Salmonella

  • Brucellosis

  • Legionella

  • Campylobacter

  • Varicella

  • Parainfluenza virus

  • Influenza virus (and vaccine)

  • Respiratory syncytial virus (RSV), rotavirus

  • Cytomegalovirus (CMV) reactivation

  • Adenoviruses 

More recently, IgAV has also been found in association with COVID-19 infections.[6][7] The virus is thought to damage blood vessels directly, leading to inflammation and immune complex formation.

This results in an influx of inflammatory mediators such as prostaglandins. The annual incidence of Henoch-Schönlein purpura in children is estimated to be between 10 to 20 cases per 100,000 children per year. In most cases, Henoch-Schönlein purpura (HSP) is a self-limiting condition, and management focuses on supportive care and symptom relief.

Supportive Care

  • Rest and Hydration: Ensuring adequate rest, especially during periods of significant pain or fatigue, is important.

    Palpable purpura can appear in many different patterns . The majority of children are aged younger than 10. Adults have a greater risk of developing chronic kidney disease.

Complications may include:

  • Bleeding inside the body
  • Blocking of the intestine (in children)
  • Kidney problems (in rare cases)

Contact your provider if:

  • You develop symptoms of IgA vasculitis, and they last for more than a few days.
  • You have colored urine or low urine output after an episode.

Immunoglobulin A vasculitis; Leukocytoclastic vasculitis; Henoch-Schönlein purpura; HSP; IgA vasculitis - Henoch-Schönlein purpura

Dinulos JGH.

Hypersensitivity syndromes and vasculitis. Pediatric Health Med Ther.

  • Causes ▾: Exact cause unknown; likely triggered by infections (e.g., upper respiratory), medications, or vaccinations, involving IgA antibody deposition. This picture shows a denser distribution with a sharp demarcation caused by what is known as Koebner’s Phenomenon (Minor trauma, such as the elastic band in one’s sock, can cause such a pattern).

    2009 Oct 01;80(7):697-704. 2020;11:572754. These potential triggers suggest that Henoch-Schönlein purpura (HSP) is likely a multifactorial disease in individuals with a genetic predisposition.

    • Infections: Upper Respiratory Tract Infections (URTIs) is the most frequently reported preceding event, occurring in up to 50-75% of cases.

      [PMC free article: PMC5902957] [PubMed: 29661187]

      11.

      Sugino H, Sawada Y, Nakamura M. IgA Vasculitis: Etiology, Treatment, Biomarkers and Epigenetic Changes. 2014 Nov;60(11):1007-10. Approximately 50% of patients develop renal manifestations, with less than 1% progressing to end-stage renal failure.[13] Death from IgA vasculitis is rare; however, renal disease is the most common cause of morbidity and mortality in patients with the disorder.[5]

      Joints

      Approximately 15% of patients with IgA vasculitis present with arthritis as the initial symptom, and overall arthralgia or arthritis occurs in 75% of children with the disorder.[9] Patients often present with painful, swollen joints that most commonly involve the knees, ankles, hands, and feet.

      Steroids, however, do not appear to improve the rash; although usually, over weeks to months, the recurrent bouts of purpura usually resolve on their own.

      Living With HSP

      Supportive care may involve a short course of prednisone or an NSAID, such as naprosyn or ibuprofen, if the kidneys are not involved. The rash may also be itchy.

      Figure 11.


      Palpability variable.Social history and physical exam findings concerning for abuse.Drug ReactionsPurpuric rash temporally related to drug exposure, may have other allergic-type symptoms.History of new medication, resolution with drug discontinuation.

      Treatment and Management of Henoch-Schönlein Purpura (HSP)

      The approach largely depends on the severity of the symptoms and the extent of organ involvement, particularly renal involvement.

      Vesiculobullous lesions These are also more common with the adult form of HSP.

      Adults are more prone to permanent kidney damage. As a result, patients often present with a diverse range of clinical manifestations, including gastrointestinal complaints, palpable purpura, arthralgias, and renal involvement.