Henoch Schönlein purpura entities
Clinic
- HSP also known as IgA vasculitis, is a disease of the , mucous membranes, and sometimes other organs
- Palpable purpura often with joint pain and abdominal pain.
- Hematuria and proteinuria, but this usually goes unnoticed, the kidney involvement proceeds to chronic kidney disease.
- HSP is often preceded by an infection, such as a throat infection.
- HSP is a systemic vasculitis and is characterized by deposition of immune complexes containing the antibody immunoglobulin A (IgA); the exact cause for this phenomenon is unknown.
Signs / Symptoms
- More severe case of HSP on child's foot, leg, and arm
- Purpura, arthritis, and abdominal pain are known as the "classic triad"
- Purpura 100%, Arthralgia / Arthritis 80%, and Abdominal pain 62%.
- Gastrointestinal hemorrhage 33% maybe due to intussusception.
- Abdominal pain is colicky in character, and may be accompanied by nausea, vomiting, constipation or diarrhea. There may be blood or mucus in the stools. The joints involved tend to be the ankles, knees, and elbows, but arthritis in the hands and feet is possible; the arthritis is nonerosive and hence causes no permanent deformity.
- 40% have evidence of kidney involvement, mainly hematuria, More than half also have proteinuria which in one eighth is severe enough to cause nephrotic syndrome. While abnormalities on urinalysis may continue for a long time, only 1% of all HSP patients develop chronic kidney disease. Hypertension may occur.
- Problems in other organs, such as CNS/ Spinal cord and lungs may occur, but is much less common
Of the 40% of patients who develop kidney involvement, almost all have evidence (visible or on urinalysis) of blood in the urine. More than half also have proteinuria (protein in the urine),
Pathophysiology
Henoch–Schönlein purpura is a small-vessel vasculitis in which complexes of immunoglobulin A (IgA) and complement component 3 (C3) are deposited on arterioles, capillaries, and venules (hence it is a type III hypersensitivity reaction). The activation of the alternative complement pathway results in the deposition of IgA aggregates or IgA complexes in target organs (with deposition of C3). This leads to the production of inflammatory mediators, including vascular prostaglandins like prostacyclin, which may play a key role in the development of IgAV and its organ-specific clinical manifestations. As with IgA nephropathy, serum levels of IgA are high in HSP and there are identical findings on renal biopsy; however, IgA nephropathy has a predilection for young adults while HSP is more predominant among children. Further, IgA nephropathy typically only affects the kidneys while HSP is a systemic disease. HSP involves the skin and connective tissues, scrotum, joints, gastrointestinal tract and kidneys. The genetic basis remains unclear except for involvement of the human leukocyte antigen region of the genome. It is hypothesized to involve autoimmunity triggered by infections. Streptococcus strains and Parainfluenza virus are the most commonly associated pathogens, and in children Human Parvovirus B19 is a frequent viral trigger
Entities
- Pethechia / Purpura
- Artritis
- Abdominal pain /Nauseas / Constipation or Diarrhea
- Nephritis
Miasms
Since vasculitis is the central pathophysiology of HSP, B19 is the best miasm.
- ↑ https://www.banglajol.info/index.php/JOM/article/view/19692#:~:text=Henoch%2DSch%C3%B6nlein%20Purpura%20(HSP),Hepatitis%20B%20viral%20infection.
- ↑ Cioc AM, Sedmak DD, Nuovo GJ, Dawood MR, Smart G, Magro CM. Parvovirus B19 associated adult Henoch Schönlein purpura. J Cutan Pathol. 2002 Nov;29(10):602-7. doi: 10.1034/j.1600-0560.2002.291006.x. PMID: 12453298.
- ↑ Karakayali B, Yilmaz S, Çakir D, Günes PG, Güven S, Islek I. Henoch-Schonlein purpura associated with primary active Epstein-Barr virus infection: a case report. Pan Afr Med J. 2017 May 11;27:29. doi: 10.11604/pamj.2017.27.29.10481. PMID: 28761605; PMCID: PMC5516656.