Henoch-Schonlein Purpura

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Retrieved

2022-04-26

Source

Gard

Trials

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Genes

C3, PLAU, SPAST, ATL1, KIF5A, MEFV, HLA-DRB1, HSP90B2P, SPG7, REEP1, SPG11, CYP7B1, ZFYVE26, CYP2U1, IGAN1, IGHA1, NIPA1, CXCL8, ACE, HSPD1, CAPN1, CRP, SPART, IL10, WASHC5, DDHD2, DDHD1, BSCL2, KIF1A, TFG, TNF, CCL2, FA2H, ERLIN2, PLP1, IL6, REN, NOS3, SOD1, PRDX5, MIF, VCP, HAVCR1, VEGFA, ALDH18A1, IL17A, KIF1B, PTHLH, MAD2L1BP, AGT, GBA2, PDXP, HSP90AA1, HLA-B, IL1B, IFT122, TLR4, PAX2, BICD2, ARL6IP1, SFXN1, RBM45, KIF1C, DNM2, CTLA4, CD28, CCL5, CPT1C, ATP13A2, SLC2A1, SLC33A1, IGFALS, PTPN22, SPG16, UBAP1, L1CAM, DNM1L, ARHGEF5, TIMELESS, AP5Z1, ITPR1, PLA2G6, SPG27, ABCB6, TECPR2, WG, FARS2, AP4B1, MIR33A, TUBB4A, HT, DNAJB1P1, C9orf72, LRPPRC, PNPLA6, IL18BP, CIC, AFG3L2, ZFYVE27, MMP26, ATAD3A, REEP2, C1GALT1, HSPA14, SCYL1, SEC24D, ICOS, NAAA, ALS2, CXCL16, POU2F3, IL21, PLD3, DAPK2, TARDBP, KIF13B, UBA5, RNF170, PITPNM3, DPY30, HAVCR2, IL17F, IL22, ASIC2, ASIC3, FXN, GATA3, GPT, GRM1, HLA-A, HMGB1, HSPA1A, HSPA1B, HSPA2, HSPA4, DNAJB1, ICAM1, IFNG, IL1A, IL1RN, IL6R, B4GALNT1, FCGRT, AP4M1, FAT2, AGTR1, ALB, ATP1A1, BCL2, BCL6, CXCR5, C5AR1, CFTR, COL4A5, CRMP1, CSK, DNMT1, EPHB2, ESR1, F2, IL6ST, IL18, CXCL10, ITGB2, RNF6, ATXN2, CXCL5, CX3CL1, SLC1A2, PARP1, SPTAN1, TIMP4, TLR2, TP53, SCGB1A1, UTRN, XRCC1, DENR, DLEU2, MAPK3, MAPK1, PON1, ATXN3, KCNA2, KIF5B, KIF5C, KRT10, MBL2, MGAT5, MMP3, PF4, MMP9, MTHFR, TRNT, NEFL, NM, NOS2, SPG36

Drugs

Budesonide (oral use) ( JORVEZA ), Recombinant human monoclonal antibody against mannan-binding lectin-associated serine protease-2

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Henoch-Schonlein purpura (HSP), also called immunoglobulin A vasculitis (IgAV), is a vascular disease that primarily affects small blood vessels. The disease is characterized by abnormal deposits of immunoglobulin A (an antibody) in the blood vessels, leading to their inflammation (vasculitis). The small vessels of the skin, joints, kidneys, and digestive organs are particularly involved. Signs and symptoms usually begin suddenly (and progress over days) and may include purple-colored spots on the skin (purpura); joint pain; and gastrointestinal problems such as abdominal pain, nausea, bloody stools, and rarely, severe complications requiring surgery. People with HSP may also develop glomerulonephritis (injury to the kidneys caused by inflammation) and poor kidney function, which may result in swelling of parts of the body or face (edema), and blood and protein in the urine (hematuria and proteinuria). Most cases of HSP occur in children and go away without causing serious or long-term health problems. Less commonly, the disease affects adults and may be more severe, leading to chronic kidney disease and kidney failure. The cause of HSP is not completely understood, but research indicates that genes (especially those involved in regulating the immune system) may play a key role in predisposing a person to HSP, as well as its severity. However, while genes may increase the risk of developing the disease (and in some cases more than one family member has HSP), the disease itself is not inherited. Environmental “triggers” such as foods, infections, or medications may also play a role in the onset of the disease. The diagnosis of HSP may be made based on symptoms, blood and urine tests, imaging studies, and/or a biopsy of the skin or kidney. Most cases go away within several weeks without treatment. When needed, treatment aims to relieve symptoms and may include medications for pain and inflammation. People with chronic kidney involvement or advanced kidney disease may require immunosuppressive medications, hemodialysis, or kidney transplantation. The long-term outlook depends on the extent of kidney involvement. Rarely, HSP is fatal due to kidney complications. In some cases, the disease recurs, sometimes more than once.