Schönlein-Henoch Purpura

Schönlein-Henoch purpura

Author: Professor Seza Ozen1 Creation Date: March 2003

Scientific Editor: Professor Loïc Guillevin

1Department of Paediatrics, Hacettepe University Faculty of Medicine, Sihhiye, 06100, Ankara, Turkey. [email protected]

Abstract Keywords Disease name and synonyms Diagnosis Differential Diagnosis Incidence Laboratory investigations Etiology Genetics Diagnostic methods Management and treatment Unresolved questions Key words References

Abstract Schönlein-Henoch purpura (HSP) is a systemic vasculitis that affects vessels of a small calibre. Diagnosis is clinically established when at least 2 of the following 4 criteria are present: palpable purpura, age less 20 years at disease onset, bowel angina, and wall granulocytes on biopsy. HSP incidence in children is at least 135 per million, about 100 times more than in adults. The characteristic presentation of this syndrome is a triad of purpura, abdominal pain and arthritis. The typical skin feature is non- thrombocytopenic palpable purpura, the cutaneous lesion is a leukocytoclastic angiitis of the small vessels. Inflammation of visceral blood vessels and gut ischemia result in abdominal pain and bloody stools, possible intussusceptions, and very rarely perforation. Arthritis is especially common in children. Renal involvement occurs in almost half of the patients and usually presents as microscopic hematuria with varying degrees of proteinuria. Despite being one of the most common vasculitides, definite data on etiology and pathogenesis are still missing. Several different bacterial organisms have been involved as the initiating factors of the disease. HSP is not a monogenic disease. Certain genetic polymorphisms have been suspected to affect disease course. Therapy is administered on a case-to-case basis and treatment of the clinical manifestations generally consists in non steroidal anti-inflammatory agents and immunosuppressants.

Keywords Systemic vasculitis, palpable purpura, bowel angina, wall granulocytes, onset in the childhood

Disease name and synonyms Diagnosis • Henoch- Schönlein purpura (HSP) Diagnosis of HSP is established when at least 2 • Schönlein-Henoch purpura, of the following 4 criteria are present: • Anaphylactoid purpura • Palpable purpura • Rhumatoid purpura • Age <20 years at disease onset • Bowel angina • Wall granulocytes on biopsy

Ozen S. Henoch-Schonlein purpura. Orphanet encyclopedia, April 2003. http://www.orpha.net/data/patho/GB/uk-HSP1.pdf 1

These criteria were issued by the American other areas as well. The color of the lesions College of Rheumatology in 1990. However, tends to change from red to purple and brown. diagnosis of HSP is usually made by Edema of the extremities and scalp is also pediatricians only when recognizing typical typical. purpura (Figure 1). The disease may also present in adults. Gastrointestinal tract Inflammation of visceral blood vessels and gut ischemia result in abdominal pain and bloody stools, possible intussusceptions, and very rarely perforation.

Musculo-skeletal system Arthritis is especially common in children. However, in a recent study only 23% of the adults had arthritis. Arthralgia may also occur.

Kidney Renal involvement occurs in almost half of the patients and usually presents as microscopic hematuria with varying degrees of proteinuria. Nephrotic syndrome may occur. In children renal failure is rare, whereas it is more common in Differential Diagnosis adults, as high as 10%. Other vasculitides need to be excluded. Others Patients with polyarteritis nodosa and Wegener’s Central nervous system, pulmonary, cardiac and granulomatosis (WG) may present with purpura testicular involvement has been very rarely and leukocytoclastic vasculitis of the skin. observed. Therefore these two entities should not be mistaken for HSP. Laboratory investigations Other clinical signs, such as severe kidney and The complete blood count is normal except for other organs involvement, can also be found in mild leukocytosis in some cases. Urinalysis must microscopic polyangiitis (polyarteritis), WG, as be performed, and when normal, followed for up well as Churg-Strauss syndrome and to 3 months, after which renal disease is unlikely cryoglobulinemic vasculitis in adults. Diagnosis to occur. In patients with renal disease, the of these different entities will only be made degree of proteinuria should be determined and relying on other disease-specific features and renal function tests performed. biopsy of affected organs. In patients with severe abdominal pain, an In rare atypical cases -without palpable lesions-, ultrasound is helpful to delineate whether an a complete blood count may be required to intussusception is present. Stools should be exclude thrombocytopenia. examined for visible or occult blood. Ig A levels may be elevated in 1/3-1/2 of the Incidence patients. HSP occurs mainly in children: incidence in children is at least 135 per million, about 100 Etiology times more than in adults. Ninety percent of Despite being one of the most common patients are less than 10 years. vasculitides, definite data on etiology and Clinical features pathogenesis are still missing. Gastrointestinal involvement is an early Several different bacterial organisms have been symptom of the disease whereas renal disease suspected to be involved as the initiating factors is a late symptom. Disease course is more of disease. For example, one 3-year prospective severe in adults. The characteristic presentation study was carried out by Al-Sheyyab et al. in of HSP in children is a triad of purpura, order to examine the association of streptococci abdominal pain and arthritis with the disease. Antistreptolysin O titre positivity was associated with a 10-fold increase in the risk Skin of HSP. IgA1 plays a critical role in the The typical feature of the disease is non- pathogenesis of HSP and abnormalities in the thrombocytopenic palpable purpura, the glycosylation of the hinge region of IgA1 may be cutaneous lesion is a leukocytoclastic angiitis of associated with the disease. IgA is the principal the small vessels. This rash is most prominent antibody in the respiratory system for defense on pressure-bearing areas, especially in the lower extremities and buttocks, but may occur in

Ozen S. Henoch-Schonlein purpura. Orphanet encyclopedia, April 2003. http://www.orpha.net/data/patho/GB/uk-HSP1.pdf 2

against microbial agents. HSP is often preceded Unresolved questions by an upper respiratory tract infection. No evidence-based data are available to show whether the use of corticosteroids has an effect Genetics on renal outcome or on the final outcome of the HSP is not a monogenic disease. Certain gastrointestinal involvement. Definite data on the genetic polymorphisms have been suspected to pathogenesis, the factors leading to renal affect disease course. involvement and preventive measures (if any) In a Spanish study, a certain polymorphism of are still missing. Data are also needed to define the IL-1RA (Interleukin 1 Receptor Antagonist) factors predicting severe renal involvement. gene was associated with severe renal Optimal therapy for mild and severe renal involvement. In another study HLA-B35 gene involvement is yet to be determined. has been suggested to encode renal complications. References Allen DM, Diamond LK, Howell DA. Diagnostic methods Anaphylactoid purpura in children. Am J Dis HSP is diagnosed clinically. Skin biopsy is rarely Child 1960; 99: 833-854. –if ever-, indicated. When renal biopsy is Al-Sheyyab M, Batieha A, el-Shanti H, Daoud A. performed the characteristic finding is IgA Henoch-Schonlein purpura and streptococcal deposition mainly in the glomerular mesangium; infection: a prospective case-control study. Ann typically focal and segmental glomerulosclerosis Trop Paediatr 1999; 19, 253-5. is observed. Foster BJ, Bernard C, Drummond KN, Sharma AK. Effective therapy for severe Henoch- Management and treatment Schonlein purpura nephritis with prednisone and Joint pain responds well to non steroidal anti- azathioprine: a clinical and histopathologic study. inflammatory agents. Cutaneous manifestations J Pediatr 2000; 136: 370-5. are often self-limited, but may have a relapsing Garcia-Porrua C, Calvino MC, Llorca J, Couselo pattern. For chronic cutaneous lesions different JM, Gonzales-Gay MA. Henoch-Schonlein treatment modalities have been tried such as purpura in children and adults: clinical colchicine and aspirin, dapsone and even differences in a defined population. Semin systemic corticosteroids. Arthritis Rheum 2002; 32: 149-56. The optimal management of gastrointestinal and Koskimies O, Rapola J, Savilahti E, Vilska J. renal involvement has not been determined yet. Renal involvement in Schönlein–Henoch Abdominal pain is common and usually self- purpura. Acta Pediatr Scand 1974; 63: 357–63. limited. Parents should be informed in case the Mollica F, Li Volti S, Garozzo R, Russo G. child develops severe pain. For severe Effectiveness of early prednisone treatment in abdominal pain uncontrolled studies support a preventing the development of nephropathy in short course of corticosteroids. An oral dose of anaphylactoid purpura. Eur J Pediatr1992; 151: prednisone of 1 mg/kg/day tapered after 140-4. resolution of symptoms is usually sufficient. The Niaudet P, Habib R. Mehtylprednisolone pulse major complications of gastrointestinal therapy in the treatment of severe forms of involvement are massive bleeding, perforation or Schonlein-Henoch purpura nephrits. Pediatr intussusception. Nephrol 1998; 12: 238-243. Patients with mild renal involvement such as Oner A, Tinaztepe K, Erdogan O. The effect of those with hematuria and mild proteinuria only, triple therapy on rapidly progressive type of require neither renal biopsy nor Henoch-Schonlein nephritis. Pediatr Nephrol immunsuppressive treatment. They should be 1995; 9: 6-10. followed-up closely for the possible development Ozen S. The spectrum of vasculitis in children. of more severe renal disease, however. Best Pract Res Clin Rheumatol 2002; 16: 411- Corticosteroids have not been shown to alter the 25. prognosis in patients with mild renal disease. In Padeh S, Passwell JH. Successful treatment of those with severe renal disease, with nephrotic chronic Henoch-Schonlein purpura with range proteinuria or impaired renal function, a colchicine and aspirin. Isr Med Assoc J 2000; 2: renal biopsy and high-dose corticosteroids with 482-3. or without immunosuppressants have been Saulsbury FT. Henoch-Schönlein Purpura. Curr suggested. If the crescent formation exceeds Opin Rheumatol 2001; 13; 35-40. 50%, an aggressive approach is suggested with Szer IS. Henoch-Schönlein purpura: when and 3 pulses of methylprednisolone followed by at how to treat. J Rheumatol 1996; 23: 1661-5. least 3 months of oral corticosteroids (1.5 mg prednisone/kg/day) and oral cyclophosphamide (2 mg/kg/day).

Ozen S. Henoch-Schonlein purpura. Orphanet encyclopedia, April 2003. http://www.orpha.net/data/patho/GB/uk-HSP1.pdf 3