153T. Zuberbier et al. (eds.), Urticaria and Angioedema,DOI: 10.1007/978-3-540-79048-8_Appendix, © Springer Verlag Berlin Heidelberg 2010

Appendix

Recommended Treatment Algorithm for Chronic Urticariawhich is part of the New 2009 International Guideline on Urticaria

The guideline is the result of a consensus conference and discussions between an expert panel and more than 200 practicing specialists in the fi eld. The stepwise approach in the algorithm is based on the evidence level available for the different drug interventions but is also taking into consideration the safety profi le and the costs. The following consider-ations have been made for the different levels:

Comments on procedure on algorithm for chronic urticaria

First Level: High quality evidence

Low cost (worldwide availability also in developing countries mostly cheaper than old • sedating antihistamines)Very good safety profi le• Very good evidence for efficacy•

If symptoms persistafter 2 weeks

If symptoms persistafter 1-4 weeks

If symptoms persistafter 1-4 weeks

Non sedating H1-antihistamine (nsAH)

nsAH updosing (up to 4x)

Add Leukotriene antagonist or change nsAH

Exacerbation: Systemic Steroid (for 3 – 7 days)

Add Ciclosporin A, H2-antihistamine, Dapsone, Omalizumab

Exacerbation: Systemic Steroid (for 3 – 7 days)

154 Appendix

Second level: low-quality evidence

Low cost• Good safety profi le• Good evidence for efficacy•

Third level: very-low-quality evidence

Low to medium–low cost • G• ood safety profi le Insufficient or no evidence for efficacy in high-quality RCT•

Fourth level:

Ciclosporin A:• Medium to high cost –Moderate safety profile –Moderate level of evidence for efficacy –

H2-Antihistamine:• Low cost –Good safety profile –Very low level of evidence for efficacy –

Dapsone:• Low cost –Medium level of side effects –Low level of evidence for efficacy –

Anti-IgE:• High cost –Good safety profile –Low level evidence for good efficacy –

155

Index

AAdhesion molecules, 11, 28Aetiology, 3, 10, 11, 39–40, 130, 137Aggravating factors, 10, 20, 52–53Allergens, 3, 15–17, 54, 64, 93,

118, 137, 149Allowed, 143, 145Angiooedema, 9–11, 13–15, 21, 25–31,

37, 38, 42, 45–47, 58, 81, 84, 86, 130, 136, 137, 141

Antagonists, 2–5, 42, 54, 83, 85, 105, 123, 124, 130, 134, 135, 138

Antihistamines, 1, 4–5, 15, 21, 38, 40–42,45, 52–54, 59–61, 68, 69, 73, 77, 78, 81, 85–87, 94, 105, 114, 117, 120, 123, 129, 132–138, 143, 145

Associations, 4, 20, 21, 30, 35, 73, 98, 99, 102–104, 112, 113, 119, 135

ASST. See Autologous Serum Skin TestAutoantibodies, 1, 4, 5, 9, 11, 16–21,

28, 54, 97, 98, 111, 114Autoimmune urticaria, 4, 12, 17–20Autoinfl ammation, 97–106Autologous serum skin test (ASST), 5, 19,

21, 48–49, 54, 67, 136, 142–144, 151

Autoreactivity, 1, 28, 45, 48–49, 52, 54, 142–144

BBacteria, 50 , 142Basic and advanced diagnostics, 52Blocking, 52, 54Bradykinin, 1, 9, 14, 15, 117,

119–121, 124, 15

CCAPS. See Cryopyrin-associated periodic

syndromeCauses, 10, 15–16, 28–31, 34, 37,

39–40, 50, 53, 76–77, 113Causes and triggers, 48Causes mast cell mediator release, 15–16Cellular and molecular basis, 2Chronic “idiopathic” urticaria (CIU), 1, 3–5,

11, 13, 35, 59–60Chronic urticaria (CU), 1, 3–5, 9–11, 13,

14, 16–20, 26–30, 33–36, 41, 45–54, 59, 61, 110, 113, 117, 120, 133–136, 141–144, 146, 149, 151

CIU. See Chronic “idiopathic” urticariaClassifi cation, 5, 10, 25–31, 46, 49,

64, 68, 77, 81, 112, 118, 130Clinical, 2, 9–11, 15–21, 25, 29, 33, 35,

38–39, 40, 51, 53, 58–61, 68, 77, 91, 99, 102–104, 115, 118, 120, 122–124, 130, 142, 149

Clinical approach, 122–123Clinical aspects, 38–39Clinical features, 10, 60–61, 66, 68,

73–74, 83–84, 99, 102,111–112, 114, 120–121

Clinical fi ndings, 58, 93Clinical picture, 45, 47, 74, 130, 137Cold stimulation test (CST), 66Cold urticaria, 63–69Complement, 1, 10, 14, 18, 50, 67, 104,

109–113, 119, 124Concept model, 19–21Confi rmation, 66–67, 84, 102, 103, 115

156 Index

Core message, 1, 9, 25, 33, 37, 45, 57, 59, 63, 73, 81, 91, 97, 109, 117, 129, 141

Corticosteroids, 41–42 , 68, 93, 114, 115, 117, 120, 132, 133, 135, 136

Course, 20, 30, 34, 40–42, 45, 58, 68, 94, 109, 114, 132, 141, 142, 147, 94

Cyclo-oxygenase (COX), 12, 13, 118, 136COX See Cyclo-oxygenaseCryopyrin-associated periodic syndrome

(CAPS), 97, 99, 102, 103CST. See Cold stimulation testCU-Q2oL, 33, 34CU See Chronic urticariaCyclosporin A, 129, 135, 137Cysteinyl leukotrienes, 12–13, 118

DDefi nition, 5, 9, 11, 25–29, 37, 45–47, 59, 110,

123Delayed pressure urticaria (DPU), 11, 12, 14,

28, 57–61, 119, 136Diagnosis, 5, 15, 25, 26, 38, 41, 42,

57–60, 64, 66–67, 84–86, 93, 98–100, 103–105, 110, 113–115, 117, 121–122, 124, 141

Diagnostic approach, 26, 94Diagnostic evaluation, 105Diagnostic implications, 58–59, 61Diagnostics, 51–52Diary cards, 146Diet, 3, 50, 53, 133–134, 142, 144–146, 151Differential diagnosis, 26, 37–38, 84–85, 98,

100, 113–114,121–122Disease activity, 20, 29–31, 38,

49–51, 104, 142Diseases related, 30, 37Disease spectrum, 63–64, 81–82DPU. See Delayed pressure urticariaDuration, 28–31, 35, 37, 41, 42, 45, 47,

49, 58, 61, 65, 68, 69, 101, 110, 114, 121, 129, 130, 134, 137, 143, 144

E© ECARF, 26–28, 39, 121EIA. See Exercise-induced anaphylaxisEliciting factors, 28, 39, 41, 51, 45, 94, 136Elicitors, 92Endogenous, 5, 10, 19

Enigma of chronic “idiopathic” urticaria, 3–4Epidemiology, 37, 38, 47, 64, 82, 110Etiology, 91–93, 102, 104Evidence-based treatment, 130–137Exercise-induced anaphylaxis (EIA), 81–87

FFamilial cold-induced autoinfi ammatory

syndrome (FCIAS), 99Familial mediterranean fever (FMF), 99, 103,

105Familial urticarial syndromes, 98–102Family, 64, 98, 124FCIAS. See Familial cold-induced

autoinfi ammatory syndromeFirst steps, 2–3Flare, 2, 9, 12, 45, 46, 49, 58, 59, 63, 75, 83,

84, 144, 75FMF. See Familial Mediterranean FeverFood, 3, 5, 17, 21, 28, 39, 41, 42, 50,

54, 66, 69, 82, 83, 86, 92, 93, 133, 135, 136, 138, 144 –148, 151

Food elicited, 144–145, 151Food labelling rules, 145–146Forbidden, 145Frequency, 3, 4, 15, 16, 18, 20,

28–31, 87, 93, 130

GGenotype, 102–103Genotype-phenotype disparity, 102–103Glucocorticosteroids, 133–135

HHAE. See Hereditary angioedemaHageman factor, 14, 124H1 Antihistamines, 4, 5, 81, 86, 87,

129, 132, 134–138, 134H2-Antihistamines, 134Health-related quality of life (HRQL),

33–36Heat urticaria, 63–69, 85, 137Hereditary angioedema (HAE), 117, 119–124HIDS. See Hyperimmunoglobulin D syndromeHistamine, 2, 9, 12, 17, 30, 59, 65, 143Histamine liberators, 92Histamine release, 65Histamine-releasing autoantibodies, 4, 11,

17, 21Histology, 28

Index 157

Histopathologic fi ndings, 110Histopathology, 10–11, 60, 74History, 1–5, 57–58, 60HRQL. See Health-related quality

of life (HRQL)Hyperimmunoglobulin D syndrome

(HIDS), 99, 105

IIgE, 1, 3–5, 9, 15–18, 28, 39–41, 48, 54,

65, 6876, 78, 86, 91, 92, 94, 98, 133, 137, 144

Immunological stimuli, 9, 17–18Infection, 3, 5, 10, 17, 20, 28, 37,

40–42, 45, 48, 50, 53, 54, 59, 64, 65, 67, 68, 111, 114, 132, 133, 137

Infl ammatory cells, 9, 11, 13–14, 40Inhibition spectrum, 75, 76Inhibitors, 5, 9, 10, 12–15, 53, 61, 105,

117–119, 121, 122–124, 130, 136Ins and outs, 145–146Intolerance, 28, 39, 45, 50, 53, 54, 135, 138,

144Introduction, 2, 63, 81Investigations, 113–114, 141, 151Ischemic heart disease (IHD), 34

LLaboratory assays, 18–19Leukotriene antagonists, 54, 135Loratadine, 40, 41, 61, 134

MMast cell, 1, 9, 25, 40, 47, 63, 74,

82, 92, 111, 130, 143 activation, 12, 48, 49, 50, 52–54, 111liberators, 12, 16, 18mediators, 3, 12–13, 15–16, 52–54, 63secretion, 15–18Mediators, 12–13, 54, 65Medical history questionnaire, 149Methods, 60, 94Molecular pathways, 97, 98

NNatural course, 40–41Neonatal-onset multisystem infi ammatory

disease (NOMID), 99Neutrophilic urticaria, 98, 100, 104

NOMID. See Neonatal-onset multisystem infi ammatory disease

Nonfamilial autoinfl ammatory syndromes, 104

Notthingam health profi le (NHP), 34

OOther therapeutic options, 136

PParasites, 50Pathogenesis, 3, 4, 11, 12, 13, 17, 20, 28,

47–48, 65, 74–77, 82–83, 91–93

Pathophysiology, 40, 99, 111, 120, 124Patients, 1, 9, 26, 33, 38, 45, 58, 63, 73, 81,

92, 99, 109, 117, 129, 141Perspectives, 2, 19, 34, 36, 123–124Pharmaceuticals, 93Pharmacological treatment, 68Phenotype, 13, 102–103Physical urticaria, 4, 10, 20, 29, 35,

37, 45, 58, 61, 64, 73, 81, 83, 130, 136–137

Polygenic etiology, 104Prednisolone, 40–42, 61, 85, 132 Prevalence, 38–40, 47, 50, 64, 82, 103, 110,

118, 119Prick test, 41, 86, 93, 94, 144, 149–151Products, 17, 93, 146Prognosis, 67, 69, 77, 85–86, 94, 114, 119Prophylaxis, 86, 123, 124Pruritus, 5, 30, 31, 34, 35, 51, 81,

85, 86, 114, 130Pseudoallergen-low, 134, 144Pseudoallergens, 3, 10, 16, 18, 20,

21, 50, 151Pseudoallergic, 18, 39

QQuality of life questionnarie, 33Questionnaire, 33–35, 38, 146–149, 151Questions, 3, 51, 58, 142, 151Questions that should be asked, 51

RRecent advances, 123–124Receptor ligand, 48, 49Recommendation, 26, 54, 129, 131, 132Removing underlying causes, 52–53

158 Index

SScore, 30, 31, 34, 35, 51, 142, 151SD. See Symptomatic dermographismSeverity, 11, 13, 17, 20, 33, 38, 59,

61, 63, 64, 66, 77, 112, 113, 123, 141, 146, 151

Skin prick test, 144, 149–151SOPs. See Standard operating proceduresSpecial, 2, 29, 37, 68, 69, 93, 137Spontaneous acute urticaria, 11, 130–133Spontaneous chronic urticaria, 19, 133Spontaneous urticaria, 10, 11, 63, 64, 69, 83,

130–136Standard operating procedures (SOPs),

141–151Subtypes, 25, 28, 29, 31, 63, 112, 117, 118,

120, 122–124, 129–132, 136–138, 141

Symptomatic dermographism (SD), 17, 57–61Symptomatic treatment, 45, 53, 54, 69, 132,

134, 138, 142, 143Symptoms, 20, 26, 28–31, 34, 35, 38, 40–42,

45, 47, 50, 51, 53, 54, 58, 61, 66, 67–69, 74, 77, 81, 82, 84–87, 93, 94, 99, 104, 110, 112, 120, 121, 130, 133, 135, 142, 144, 146, 147

Systemic manifestations, 112–113

TTake home pearls, 5, 21, 31, 36, 42, 54, 59,

61, 69, 78, 87, 94, 105, 114–115, 124, 137–138, 151

Temperature, 2, 29, 63, 65–69, 81–83, 85–87, 137

Testing, 19, 50, 59–61, 67, 84, 104, 114, 122, 124, 142–144, 149

Tests, 3, 13, 18–19, 41, 50, 67, 84, 86, 101, 104, 113, 122, 124, 134, 144

Therapy, 33, 52–54, 68, 94, 104, 114, 118, 123, 124, 129–138, 143

Threshold, 19–21, 51, 63, 65, 66–68, 86Thyroid hormones, 135Tolerance induction, 63, 68–69

TRAPS. See Tumor necrosis factor receptor-associated periodic syndrome

Treatment, 1–5, 17, 20, 25, 30, 33–36, 40–42, 45, 53, 54, 58–61, 63, 65, 67–69, 77, 84–87, 94, 97, 98, 101, 105, 106, 114, 115, 117, 119–121, 123, 124, 129–138, 141–143, 145, 146

Treatment advice, 130Triggers, 10, 29, 48, 49, 51–53,

85, 93, 147, 151Tumor necrosis factor receptor-associated

periodic syndrome (TRAPS) , 99, 103, 105

Types, 1, 12, 21, 28, 29, 34, 37, 69, 76, 77, 91, 97, 112, 119, 120, 124, 130

UUAS. See Urticaria activity scoreUltraviolet (UV) light, 59, 73, 77,

78, 113, 115Urticaria activity score (UAS), 51, 142, 151Urticaria-calendar, 147Urticaria episode protocol, 148Urticarial syndromes, 97–106Urticaria not implicating, 14–15UVA, 74, 75, 77, 78UVB, 74, 77, 78, 85

VViruses, 40, 50, 114

WWealing, 12, 37, 40, 41, 57–59, 73–77, 130Weals, 9–13, 15, 25, 26, 29–31, 37, 38,

42, 45, 47, 51, 58, 73, 74, 76, 81–85, 117–120, 122–124, 135, 136, 141–143

Without weals, 15, 74, 117–119, 122–124