0
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What Is Schönlein-Henoch Purpura, and Why Should We Care?

Warren W. Piette, MD
[+] Author Affiliations

Department of Dermatology University of Iowa College of Medicine Iowa City, IA 52242


Arch Dermatol. 1997;133(4):515-518. doi:10.1001/archderm.1997.03890400121019
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THE STUDY by Tancrede-Bohin et al1 in this issue of the Archives provides an impetus to urge closer collaboration with our nondermatologic colleagues who are working on the puzzle of vasculitis. The Chapel Hill Consensus Group, Chapel Hill, NC, and the American College of Rheumatology have provided the most prominent current classifications of primary systemic vasculitides.2-4 Many vasculitic syndromes may present in the skin, while others may be diagnosed by use of the clinical and histological pattern of cutaneous disease that is taken in context with systemic findings. It is unfortunate that neither working group included dermatologists. As a result, many dermatologists who are experts in the field of vasculitic syndromes have reservations about aspects of both classification systems.

Traditionally, Schönlein-Henoch purpura (SHP) is defined by palpable purpura, arthralgia, gastrointestinal signs and symptoms, and glomerulonephritis.5-8 Frequently characterized as a syndrome of a single episode, SHP has in fact a remarkable tendency to recur

REFERENCES

Tancrede-Bohin E, Ochonisky S, Vignon-Pennamen M-D, Flageul B, Morel P, Rybojad R.  Schönlein-Henoch purpura in adult patients: predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases . Arch Dermatol. 1997;;133:438-442.
Jennette CJ, Falk RJ, Andrassy K, et al.  Nomenclature of systemic vasculitides: proposal of an international consensus conference . Arthritis Rheum . 1994;; 37:187-192.
Jennette CJ, Milling DM, Falk RJ.  Vasculitis affecting the skin: a review . Arch Dermatol. 1994;;130:899-906.
Hunder GG, Arend WP, Bloch DA, et al.  The American College of Rheumatology 1990 criteria for the classification of vasculitis . Arthritis Rheum . 1990;;33: 1065-1067.
Allen M, Diamond LK, Howel DA.  Anaphylactoid purpura in children . AJDC . 1960;;99:833-854.
Sterky G, Thilén A.  A study on the onset and prognosis of acute vascular purpura (the Schönlein-Henoch syndrome) in children . Acta Paediatr . 1960;;49: 217-229.
Cream JJ, Gumpel JM, Peachey RDG.  Schönlein-Henoch purpura in the adult: a study of 77 adults with anaphylactoid or Schönlein purpura . QJM . 1970;; 156:461-484.
Piette WW.  Primary systemic vasculitis . In: Sontheimer RD, Provost TT, eds. Cutaneous Manifestations of Rheumatic Diseases . Baltimore, Md: Williams & Wilkins; 1996;:177-232.
Piette WW, Stone MS.  A cutaneous sign of IgA-associated small dermal vessel leukocytoclastic vasculitis in adults (Henoch-Schönlein purpura) . Arch Dermatol. 1989;;125:53-56.
Mills JA, Michel BA, Bloch DA, et al.  The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura . Arthritis Rheum . 1990;;33:1114-1121.
Saraçlar Y, Tinaztepe K, Adalioglu G, Tuncer A.  Acute hemorrhagic edema of infancy (AHEI)—a variant of Henoch-Schönlein purpura or a distinct clinical entity? J Allergy Clin Immunol. 1990;;86:473-483.
Legrain V, Lejean S, Taïeb A, Guillard JM, Battin J, Maleville J.  Infantile acute hemorrhagic edema skin: study of ten cases . J Am Acad Dermatol. 1991;;24: 17-22.
Saraçlar Y, Tinaztepe K.  Infantile acute hemorrhagic edema of the skin . J Am Acad Dermatol. 1992;;26:275-276.
Dubin BA, Bronson DM, Eng AM.  Acute hemorrhagic edema of childhood: an unusual variant of leukocytoclastic vasculitis . J Am Acad Dermatol. 1990;;23: 347-350.
Kauffmann RH, Herrmann WA, Meÿer CJLM, Daha MR, Van Es LA.  Circulating IgA-immune complexes in Henoch-Schönlein purpura: a longitudinal study of their relationship to disease activity and vascular deposition of IgA . Am J Med . 1980;;69:859-866.
van Hale HM, Gibson LE, Schroeter AL.  Henoch-Schönlein vasculitis: direct immunofluorescence study of uninvolved skin . J Am Acad Dermatol. 1986;;15: 665-670.
Giangiacomo J, Tsai C.  Dermal and glomerular deposition of IgA in anaphylactoid purpura . AJDC . 1977;;131:981-983.
Levinsky RJ, Barratt TM.  IgA immune complexes in Henoch-Schönlein purpura . Lancet . 1979;;2:1100-1103.
Hené RJ, Velthuis P, van de Wiel A, et al.  The relevance of IgA deposits in vessel walls of clinically normal skin: a prospective study . Arch Intern Med . 1986;;146:745-749.
Kuno-Sakai H, Sakai H, Nomoto Y, Takakura I, Kimura M.  Increase of IgA-bearing peripheral blood lymphocytes in children with Henoch-Schöenlein purpura . Pediatrics . 1979;;64:918-922.
Baart de la Faille-Kuyper EH, Kater L, Kooiker CJ, Dorhout Mees EJ.  IgA-deposits in cutaneous blood-vessel walls and mesangium in Henoch-Schönlein syndrome . Lancet . 1973;;1:892-893.
Tsai C, Giangiacomo J, Zuckner J.  Dermal IgA deposits in Henoch-Schönlein purpura and Berger's nephritis . Lancet . 1975;;1:342-343.
Sinniah R, Feng PH, Chen BTM.  Henoch-Schönlein syndrome: a clinical and morphological study of renal biopsies . Clin Nephrol. 1978;;9:219-228.
Baart de la Faille-Kuyper EH, Kater L, Kuijten RH, et al.  Occurrence of vascular IgA deposits in clinically normal skin of patients with renal disease . Kidney Int. 1976;;9:424-429.
Galla JH.  IgA nephropathy . Kidney Int. 1995;;47:377-387.
Sinico R, Fornasieri A, Oreni N, et al.  Polymeric IgA rheumatoid factor in idiopathic mesangial nephropathy (Berger's disease) . J Immunol. 1986;;137:536-541.
Saulsbury FT.  IgA rheumatoid factor in Henoch-Schönlein parpura . J Pediatr . 1986;;108:71-76.
Tomino Y, Sakai H, Endoh M, et al.  Cross-reactivity of eluted antibodies from renal tissues of patients with Henoch-Schönlein purpura nephritis and IgA nephropathy . Am J Nephrol. 1983;;3:315-318.
Berthoux FC, Gagne A, Sabatier JC, et al.  HLA-Bw35 and mesangial IgA glomerulonephritis . N Engl J Med . 1978;;298:1034-1035.
Conley ME, Delacroix DL.  Intravascular and mucosal immunoglobulin A: two separate but related systems of immune defense? Ann Intern Med . 1987;;106: 892-899.
Van den Wall Bakke AWL, Daha MR, Radl J, et al.  The bone marrow as production site of the IgA deposited in the kidneys of patients with IgA nephropathy . Clin Exp Immunol. 1988;;72:321-325.
Egido J, Biasco R, Lozano J, Sancho J, Garcoa-Hoyo R.  Immunological abnormalities in the tonsils of patients with IgA nephropathy: inversion in the ratio of IgA:IgG bearing lymphocytes and increased polymeric IgA synthesis . Clin Exp Immunol. 1984;;57:101-106.
Heng MCY.  Henoch-Schönlein purpura . Br J Dermatol. 1985;;112:235-240.
André C, Berthoux FC, André F, Gillon J, Genin C, Sabatier J.  Prevalence of IgA2 deposits in IgA nephropathies . N Engl J Med. 1980;;23:1343-1346.
Williams DG.  IgA nephropathy . BMJ . 1994;;308:74-75.
Sussman M, Jones JH, Almeida JD, et al.  Deficiency of the second component of complement associated with anaphylactoid purpura and presence of Mycoplasma in the serum . Clin Exp Immunol. 1973;;14:531-539.
Einstein LP, Alper CA, Block KJ, et al.  Biosynthetic deficiency in monocytes from human beings with genetic deficiency of the second component of complement . N Engl J Med. 1975;;292:1169-1171.
Gelfand EW, Clarkson JE, Minta JO.  Selective deficiency of the second component of complement in a patient with anaphylactoid purpura . Clin Immunol Immunopathol. 1975;;4:269-274.
Ault BH, Stapleton B, Rivas ML, et al.  Association of Henoch-Schönlein purpura glomerulonephritis with C4B deficiency . J Pediatr . 1990;;117:753-755.
McLean RH, Wyatt RJ, Julian BA.  Complement phenotypes in glomerulonephritis: increased frequency of homozygous null C4 phenotypes in IgA nephropathy and Henoch-Schönlein purpura . Kidney Int. 1984;;26:855-860.
Wyatt RJ, Julian BA, Galla JH.  Properdin deficiency with IgA nephropathy . N Engl J Med. 1981;;305:1097.
Welch TR, Berry A, Beischel LS.  C4 isotype deficiency in IgA nephropathy . Pediatr Nephrol. 1987;;1:136-139.
Wyatt RJ, Schneider PD, Alpers CA, Hudson EC, Julian BA.  C4B deficiency in two siblings with IgA nephropathy . Am J Kidney Dis. 1990;;15:66-71.
Wyatt RJ, Rivas ML, Schena FP, Bin J, Julian BA.  Regional variation in C4 phenotype in patients with IgA nephropathy . J Pediatr . 1990;;116:S72-S77.
Hiemstra PS, Gorter A, Stuurman ME, Van Es LA, Daha MR.  Activation of the alternative pathway of complement by human serum IgA . Eur J Immunol. 1987;; 17:321-326.
Berger J, Hinglais N.  Les depots intercapillaires d'IgA-IgG . J Urol (Paris) . 1968;; 74:694-695.
D'Amico G.  The commonest glomerulonephritis in the world . QJM . 1987;;64: 709-727.
Julian BA, Waldo FB, Rifai A, Mestecky J.  IgA nephropathy, the most common glomerulonephritis worldwide: a neglected disease in the United States? Am J Med. 1988;;84:129-132.
Yoshikawa N, Nakamura H, Ito H.  IgA nephropathy in children and adults . Springer Semin Immunopathol. 1994;;16:105-120.
Bruneau C, Villiaumey J, Avouac B, et al.  Seronegative spondyloarthropathies and IgA glomerulonephritis: a report of four cases and a review of the literature . Semin Arthritis Rheum . 1986;;15:179-184.
Peeters AJ, van den Wall Bake AWL, Daha MR, Breedveld FC.  Inflammatory bowel disease and ankylosing spondylitis associated with cutaneous vasculitis, glomerulonephritis, and circulating IgA immune complexes . Ann Rheum Dis. 1990;;49:638-640.
Jennette JC.  The immunohistology of IgA nephropathy . Am J Kidney Dis. 1988;; 12:348-352.
van de Wiel A, Valentijn RM, Schuurman HJ, Daha MR, Hene RH, Kater L.  Circulating IgA immune complexes and skin IgA deposits in liver disease: relation to liver histopathology . Dig Dis Sci. 1988;;33:678-684.
D'Amico G.  Influence of clinical and histological features on actuarial renal survival in adult patients with idiopathic IgA nephropathy, membranous nephropathy, and membranoproliferative glomerulonephritis: survey of the recent literature . Am J Kidney Dis. 1992;;20:315-323.
Chauveau D, Droz D.  Follow-up evaluation of the first patients with IgA nephropathy described at Necker Hospital . Contrib Nephrol. 1993;;104:6-13.
van Ypersele de Strihou C.  Fish oil for IgA nephropathy? N Engl J Med. 1994;; 331:1227-1229.
Donadio JV Jr, Bergstralh MS, Offord KP, Spencer DC, Holley KE, and the Mayo Nephrology Collaborative Group.  A controlled trial of fish oil in IgA nephropathy . N Engl J Med. 1994;;331:1194-1199.
Rostoker G, Desvaux-Belghiti D, Pilatte Y, et al.  High-dose immunoglobulin therapy for severe IgA nephropathy and Henoch-Schöenlein purpura . Ann Intern Med . 1994;;120:476-484.
Weiss JH, Bhathena DR, Curtis JJ, Lucas BA, Luke RG.  A possible relationship between Henoch-Schöenlein syndrome and IgA nephropathy (Berger's disease): an illustrative case . Nephron. 1978;;22:582-589.

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Tancrede-Bohin E, Ochonisky S, Vignon-Pennamen M-D, Flageul B, Morel P, Rybojad R.  Schönlein-Henoch purpura in adult patients: predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases . Arch Dermatol. 1997;;133:438-442.
Jennette CJ, Falk RJ, Andrassy K, et al.  Nomenclature of systemic vasculitides: proposal of an international consensus conference . Arthritis Rheum . 1994;; 37:187-192.
Jennette CJ, Milling DM, Falk RJ.  Vasculitis affecting the skin: a review . Arch Dermatol. 1994;;130:899-906.
Hunder GG, Arend WP, Bloch DA, et al.  The American College of Rheumatology 1990 criteria for the classification of vasculitis . Arthritis Rheum . 1990;;33: 1065-1067.
Allen M, Diamond LK, Howel DA.  Anaphylactoid purpura in children . AJDC . 1960;;99:833-854.
Sterky G, Thilén A.  A study on the onset and prognosis of acute vascular purpura (the Schönlein-Henoch syndrome) in children . Acta Paediatr . 1960;;49: 217-229.
Cream JJ, Gumpel JM, Peachey RDG.  Schönlein-Henoch purpura in the adult: a study of 77 adults with anaphylactoid or Schönlein purpura . QJM . 1970;; 156:461-484.
Piette WW.  Primary systemic vasculitis . In: Sontheimer RD, Provost TT, eds. Cutaneous Manifestations of Rheumatic Diseases . Baltimore, Md: Williams & Wilkins; 1996;:177-232.
Piette WW, Stone MS.  A cutaneous sign of IgA-associated small dermal vessel leukocytoclastic vasculitis in adults (Henoch-Schönlein purpura) . Arch Dermatol. 1989;;125:53-56.
Mills JA, Michel BA, Bloch DA, et al.  The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura . Arthritis Rheum . 1990;;33:1114-1121.
Saraçlar Y, Tinaztepe K, Adalioglu G, Tuncer A.  Acute hemorrhagic edema of infancy (AHEI)—a variant of Henoch-Schönlein purpura or a distinct clinical entity? J Allergy Clin Immunol. 1990;;86:473-483.
Legrain V, Lejean S, Taïeb A, Guillard JM, Battin J, Maleville J.  Infantile acute hemorrhagic edema skin: study of ten cases . J Am Acad Dermatol. 1991;;24: 17-22.
Saraçlar Y, Tinaztepe K.  Infantile acute hemorrhagic edema of the skin . J Am Acad Dermatol. 1992;;26:275-276.
Dubin BA, Bronson DM, Eng AM.  Acute hemorrhagic edema of childhood: an unusual variant of leukocytoclastic vasculitis . J Am Acad Dermatol. 1990;;23: 347-350.
Kauffmann RH, Herrmann WA, Meÿer CJLM, Daha MR, Van Es LA.  Circulating IgA-immune complexes in Henoch-Schönlein purpura: a longitudinal study of their relationship to disease activity and vascular deposition of IgA . Am J Med . 1980;;69:859-866.
van Hale HM, Gibson LE, Schroeter AL.  Henoch-Schönlein vasculitis: direct immunofluorescence study of uninvolved skin . J Am Acad Dermatol. 1986;;15: 665-670.
Giangiacomo J, Tsai C.  Dermal and glomerular deposition of IgA in anaphylactoid purpura . AJDC . 1977;;131:981-983.
Levinsky RJ, Barratt TM.  IgA immune complexes in Henoch-Schönlein purpura . Lancet . 1979;;2:1100-1103.
Hené RJ, Velthuis P, van de Wiel A, et al.  The relevance of IgA deposits in vessel walls of clinically normal skin: a prospective study . Arch Intern Med . 1986;;146:745-749.
Kuno-Sakai H, Sakai H, Nomoto Y, Takakura I, Kimura M.  Increase of IgA-bearing peripheral blood lymphocytes in children with Henoch-Schöenlein purpura . Pediatrics . 1979;;64:918-922.
Baart de la Faille-Kuyper EH, Kater L, Kooiker CJ, Dorhout Mees EJ.  IgA-deposits in cutaneous blood-vessel walls and mesangium in Henoch-Schönlein syndrome . Lancet . 1973;;1:892-893.
Tsai C, Giangiacomo J, Zuckner J.  Dermal IgA deposits in Henoch-Schönlein purpura and Berger's nephritis . Lancet . 1975;;1:342-343.
Sinniah R, Feng PH, Chen BTM.  Henoch-Schönlein syndrome: a clinical and morphological study of renal biopsies . Clin Nephrol. 1978;;9:219-228.
Baart de la Faille-Kuyper EH, Kater L, Kuijten RH, et al.  Occurrence of vascular IgA deposits in clinically normal skin of patients with renal disease . Kidney Int. 1976;;9:424-429.
Galla JH.  IgA nephropathy . Kidney Int. 1995;;47:377-387.
Sinico R, Fornasieri A, Oreni N, et al.  Polymeric IgA rheumatoid factor in idiopathic mesangial nephropathy (Berger's disease) . J Immunol. 1986;;137:536-541.
Saulsbury FT.  IgA rheumatoid factor in Henoch-Schönlein parpura . J Pediatr . 1986;;108:71-76.
Tomino Y, Sakai H, Endoh M, et al.  Cross-reactivity of eluted antibodies from renal tissues of patients with Henoch-Schönlein purpura nephritis and IgA nephropathy . Am J Nephrol. 1983;;3:315-318.
Berthoux FC, Gagne A, Sabatier JC, et al.  HLA-Bw35 and mesangial IgA glomerulonephritis . N Engl J Med . 1978;;298:1034-1035.
Conley ME, Delacroix DL.  Intravascular and mucosal immunoglobulin A: two separate but related systems of immune defense? Ann Intern Med . 1987;;106: 892-899.
Van den Wall Bakke AWL, Daha MR, Radl J, et al.  The bone marrow as production site of the IgA deposited in the kidneys of patients with IgA nephropathy . Clin Exp Immunol. 1988;;72:321-325.
Egido J, Biasco R, Lozano J, Sancho J, Garcoa-Hoyo R.  Immunological abnormalities in the tonsils of patients with IgA nephropathy: inversion in the ratio of IgA:IgG bearing lymphocytes and increased polymeric IgA synthesis . Clin Exp Immunol. 1984;;57:101-106.
Heng MCY.  Henoch-Schönlein purpura . Br J Dermatol. 1985;;112:235-240.
André C, Berthoux FC, André F, Gillon J, Genin C, Sabatier J.  Prevalence of IgA2 deposits in IgA nephropathies . N Engl J Med. 1980;;23:1343-1346.
Williams DG.  IgA nephropathy . BMJ . 1994;;308:74-75.
Sussman M, Jones JH, Almeida JD, et al.  Deficiency of the second component of complement associated with anaphylactoid purpura and presence of Mycoplasma in the serum . Clin Exp Immunol. 1973;;14:531-539.
Einstein LP, Alper CA, Block KJ, et al.  Biosynthetic deficiency in monocytes from human beings with genetic deficiency of the second component of complement . N Engl J Med. 1975;;292:1169-1171.
Gelfand EW, Clarkson JE, Minta JO.  Selective deficiency of the second component of complement in a patient with anaphylactoid purpura . Clin Immunol Immunopathol. 1975;;4:269-274.
Ault BH, Stapleton B, Rivas ML, et al.  Association of Henoch-Schönlein purpura glomerulonephritis with C4B deficiency . J Pediatr . 1990;;117:753-755.
McLean RH, Wyatt RJ, Julian BA.  Complement phenotypes in glomerulonephritis: increased frequency of homozygous null C4 phenotypes in IgA nephropathy and Henoch-Schönlein purpura . Kidney Int. 1984;;26:855-860.
Wyatt RJ, Julian BA, Galla JH.  Properdin deficiency with IgA nephropathy . N Engl J Med. 1981;;305:1097.
Welch TR, Berry A, Beischel LS.  C4 isotype deficiency in IgA nephropathy . Pediatr Nephrol. 1987;;1:136-139.
Wyatt RJ, Schneider PD, Alpers CA, Hudson EC, Julian BA.  C4B deficiency in two siblings with IgA nephropathy . Am J Kidney Dis. 1990;;15:66-71.
Wyatt RJ, Rivas ML, Schena FP, Bin J, Julian BA.  Regional variation in C4 phenotype in patients with IgA nephropathy . J Pediatr . 1990;;116:S72-S77.
Hiemstra PS, Gorter A, Stuurman ME, Van Es LA, Daha MR.  Activation of the alternative pathway of complement by human serum IgA . Eur J Immunol. 1987;; 17:321-326.
Berger J, Hinglais N.  Les depots intercapillaires d'IgA-IgG . J Urol (Paris) . 1968;; 74:694-695.
D'Amico G.  The commonest glomerulonephritis in the world . QJM . 1987;;64: 709-727.
Julian BA, Waldo FB, Rifai A, Mestecky J.  IgA nephropathy, the most common glomerulonephritis worldwide: a neglected disease in the United States? Am J Med. 1988;;84:129-132.
Yoshikawa N, Nakamura H, Ito H.  IgA nephropathy in children and adults . Springer Semin Immunopathol. 1994;;16:105-120.
Bruneau C, Villiaumey J, Avouac B, et al.  Seronegative spondyloarthropathies and IgA glomerulonephritis: a report of four cases and a review of the literature . Semin Arthritis Rheum . 1986;;15:179-184.
Peeters AJ, van den Wall Bake AWL, Daha MR, Breedveld FC.  Inflammatory bowel disease and ankylosing spondylitis associated with cutaneous vasculitis, glomerulonephritis, and circulating IgA immune complexes . Ann Rheum Dis. 1990;;49:638-640.
Jennette JC.  The immunohistology of IgA nephropathy . Am J Kidney Dis. 1988;; 12:348-352.
van de Wiel A, Valentijn RM, Schuurman HJ, Daha MR, Hene RH, Kater L.  Circulating IgA immune complexes and skin IgA deposits in liver disease: relation to liver histopathology . Dig Dis Sci. 1988;;33:678-684.
D'Amico G.  Influence of clinical and histological features on actuarial renal survival in adult patients with idiopathic IgA nephropathy, membranous nephropathy, and membranoproliferative glomerulonephritis: survey of the recent literature . Am J Kidney Dis. 1992;;20:315-323.
Chauveau D, Droz D.  Follow-up evaluation of the first patients with IgA nephropathy described at Necker Hospital . Contrib Nephrol. 1993;;104:6-13.
van Ypersele de Strihou C.  Fish oil for IgA nephropathy? N Engl J Med. 1994;; 331:1227-1229.
Donadio JV Jr, Bergstralh MS, Offord KP, Spencer DC, Holley KE, and the Mayo Nephrology Collaborative Group.  A controlled trial of fish oil in IgA nephropathy . N Engl J Med. 1994;;331:1194-1199.
Rostoker G, Desvaux-Belghiti D, Pilatte Y, et al.  High-dose immunoglobulin therapy for severe IgA nephropathy and Henoch-Schöenlein purpura . Ann Intern Med . 1994;;120:476-484.
Weiss JH, Bhathena DR, Curtis JJ, Lucas BA, Luke RG.  A possible relationship between Henoch-Schöenlein syndrome and IgA nephropathy (Berger's disease): an illustrative case . Nephron. 1978;;22:582-589.

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