Eosinophilic granulomatosis with polyangiitis M30.1

Authors: Prof. Dr. med. Peter Altmeyer, Alexandros Zarotis

All authors of this article

Last updated on: 31.12.2020

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Synonym(s)

Allergic granulomatosis; Allergic granulomatosis Churg-Strauss; Allergic granulomatosis with polyangiitis; Angiitis allergic granulomatous; Churg-Strauss Syndrome; CSS; EGPA; Eosinophilic granulomatosis with polyangiitis; Granulomatosis allergic; Granulomatosis eosinophils with polyangiitis; Hypersensitivity angiitis Churg and Strauss

History
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Jakob Churg and Lotte Strauss, 1951

Definition
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Rare, systemic (necrotizing) vasculitis affecting various vessel sizes (small to medium-sized), associated with intravascular and extravascular granuloma formation, bronchial asthma, and blood eosinophilia.

Churg-Strauss syndrome is clinically distinguished from other vasculitides, particularly polyarteritis nodosa (PAN), by the presence of severe (allergic) bronchial asthma (possibly together with allergic rhinitis) in combination with marked blood eosinophilia (>10%).

Furthermore there is:

  • inconstant fever
  • transient pulmonary infiltrates
  • cardiac involvement (50%) - eosinophilic granulomatous myocarditis and coronaritis, high eosinophilia, usually ANCA negative,
  • mono-polyneuropathy
  • Renal involvement in the form of"Rapid Progressive Glomerulonephritis".
  • CNS vasculitis
  • recurrent thromboembolism
  • Skin involvement (40%) - eosinophilic granulomatous dermatitis

Occurrence/Epidemiology
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Rarely. Incidence: 0.5-1.0/100,000 inhabitants/year.

Etiopathogenesis
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The pathogenesis is still unknown. As triggers, various. Inhalation allergens are discussed as triggers. Further infections, vaccinations, medication. Repeated cases have been described under Omalizumab, a monoclonal IgE antibody.

Manifestation
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Men are affected 2-3 times more frequently than women. Manifestation age 40 - 50 years (average: 44 years).

Localization
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trunk, distal extremities, rarely head, no mucous membrane infestation

Clinical features
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Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) develops in 3 phases:

  • Allergic phase with allergic asthma, allergic rhinitis and sinusitis
  • Eosinophilic phase with eosinophilic infiltrates in the lungs, heart (skin) and gastrointestinal tract
  • Vasculitic phase with peripheral neuropathy and other organ manifestations

The skin symptoms are clinically uncharacteristic and can only be assessed diagnostically in the overall view of the clinical symptoms.

They occur in about 40% of Churg-Strauss patients, in about 20% as a first manifestation and impress as cutaneous or subcutaneously located, rough, painful red plaques, nodules or ulcers, following the course of vessels close to the skin (2/3 of the cases). Erythema or plaques that are only 5.0-10.0 cm in size, are flat or figured and heal with hyperpigmentation may also occur.

Corresponding granulomas are found in numerous organs:

  • Lung (100%) initially impressing as volatile Löffler infiltrates.
  • Also spleen, heart, liver, gastrointestinal tract, genitals, muscles, kidney.

Relapsing activities are often associated with fever, arthralgia or unspecific skin reactions such as:

Laboratory
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  • Inflammation parameters increased (BSG acceleration, CRP)
  • Pronounced eosinophilia (up to 80% or 5000/μl)
  • IgE i.S. increased
  • Positive rheumatoid factor
  • Positive ANA
  • p-ANCA (in 40-60% of cases)
  • Positive circulating immune complexes
  • IgG4 frequently increased
  • Possibly increased AP .

Histology
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Necrotizing vasculitis: Picture is largely similar to Wegener's granulomatosis with tissue eosinophilia: Coexistence of leukocytoclastic vasculitis with subcutaneous arterial vasculitis. In addition, extensive extravascular palisade granulomas with central, map-like necrosis zones, many multinucleated giant cells and distinct infiltrates with eosinophilic and neutrophilic leukocytes (Churg-Strauss granulomas).

Histopathological algorithm of eosinophilic granulomatosis with polyangiitis (lowest common denominator: italic, leading symptoms:bold) varies according to Ratzinger et al. 2105
Accentuates around post-capillary venules and larger vessels in the skin and subcutis
Capillaries omitted or less strongly involved
perivascular and intramural leukocytoclasia
Damage to endothelial cells
Fibrin in/around vessel walls
Perivascular extravasation of erythrocytes
No/mild edema in the papillary dermis
Collagen degeneration with slight basophilic necrotic lesions surrounded by palisade granulomas
Significant predominant eosinophilia
Plasma cells or fibrosclerosis to a variable degree
Reorganisation due to lymphocytic vasculitis

Diagnosis
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Criteria of the American College of Rheumatology for the diagnosis of eosinophilic granulomatosis with polyangiits (if at least 4 criteria are present, the diagnosis is likely)

  1. bronchial asthma
  2. Eosinophilia(>10%) of the blood.
  3. Mono- or polyneuropathy
  4. Radiologically detectable migrating pulmonary infiltrates
  5. Acute or chronic recurrent sinusitis
  6. Bioptic detection of extravascular eosinophilia

Internal therapy
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Glucocorticoids in medium doses (1.5-2.0 mg/kg bw prednisone equivalent/day) is often sufficient. Good response in general. Symptom-adapted reduction of steroid medication over a longer period. Maintenance dose: 2.5-7.5 mg prednisolone equivalent/day.

Alternative or complementary: In severe cases a complementary therapy with cyclophosphamide according to the Fauci scheme (see Takayasu arteritis) can be given. This therapy should be continued for at least 6 months to 1 year after full remission.

Alternatively: Mepolizumab. In a randomized, multicenter, double-blind phase III study, mepolizumab was used in eosinophilic granulomatosis with polyangiitis for a period of 52 weeks (dose 300mg s.c. every 4 weeks). Under this therapy, there was a significant reduction in disease activity (reduction in recurrence rate, reduction in glucocorticoid consumption).

Experimental: Positive single observations exist of plasmapheresis treatments.

Progression/forecast
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Untreated, almost always lethal within a few years (cardiac causes); under therapy often complete remission. 5-year survival rate with optimal therapy >80%. Most frequent cause of death: cardiac failure, myocardial infarction.

Note(s)
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The CS syndrome develops in patients with chronic asthma; gradually increasing blood eosinophilia (1500-5000/ul), Löffler's infiltrates in the lungs, vasculitic infiltrates in almost all organs.

Clinic, laboratory, histology (criteria of the American College of Rheumatology -1990-)

The diagnosis is probable if 4 of the following 6 criteria are met:

  1. Asthma (difficult to treat) (with transient pulmonary infiltrates)
  2. Eosinophilia > 10% in differential blood count
  3. Mononeuropathy or polyneuropathy associated with systemic vasculitis
  4. Migrating or transient radiologically detectable pulmonary infiltrates associated with systemic vasculitis
  5. Acute or chronically recurrent sinusitis of the paranasal sinuses or radiological changes in the sense of chronic sinusitis
  6. Biopsy of vasculitis with the detection of eosinophilia in extravascular tissue.

Case report(s)
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  • The 42-year-old man with long-term bronchial asthma that is difficult to treat and seasonal allergic rhinitis (birch pollen allergy) developed painful red lumps on his chest and hips for about 16 months, which developed into 5.0-10.0 cm red, only moderately painful, bizarrely configured (apparently following the deep vascular plexus of the skin), sharply margined plaques. A recurrent course with moderate shear activity (recurrences with 1-2 new nodules every 4-6 weeks) was characteristic. The healing of the plaques was accompanied by lesional hyperpigmentation.
  • Systemic steroid administration (prednisolone between 50 and 20 mg p.o./day) was regularly necessary to treat asthma with dyspnoea. The cutaneous lesions also responded to this with high sensitivity (subsidence under hyperpigmentation, recurrence of skin lesions below a critical threshold of about 5-10 mg/day).
  • Laboratory: blood eosinophilia: 35% (absolute: 2085/ul); CRP elevation(7.5 mg/dl); IgE:1000 U/ml, BSG 30/70 mm; rheumatoid factor, ANA, MPO-p-ANCA normative. In a later control, MPO-p-ANCA could be detected with further increasing eosinophilia (30%).
  • Histology: Pronounced perivascularly oriented infiltrates of lymphocytes, histiocytes, eosinophilic and neutrophilic granulocytes and nuclear dust. Further signs of leukocytoclastic vasculitis with vasculitis of subcutaneous arteries. Isolated palisade granulomas with central necrobiosis zones.
  • Ro.thorax: The radiographically detectable infiltrates of the lung were interpreted as "pneumonia".
  • The therapy was carried out in cooperation with pneumologists with internally applied steroids (initial 100 mg prednisolone in decreasing dosage) and supplemented with cyclophosphamide according to the Fauci scheme for 1 year. This included full remission.

Literature
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  1. Churg J, Strauss L (1951) Allergic granulomatosis, allergic angiitis and periarteriitis nodosa. On J cathetus 27: 227-301
  2. Crowson AN et al (2003) Cutaneous vasculitis: a review. J Cutan catheter 30: 161-173
  3. Drage LA (2002) Evidence for pathogenic involvement of eosinophils and neutrophils in Churg-Strauss syndrome. J Am Acad Dermatol 47: 209-216
  4. Gioffredi A et al (2014) Eosinophilic granulomatosis withpolyangiitis: an overview. Front Immunol 5:549
  5. Groh M et al(2015) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) ConsensusTask
    Force recommendations for evaluation and management. Eur J Internal Med 26:545-553
  6. Gruer P, Bold T, Vilardo L (2001) Case 30-2000: Churg-Strauss syndrome. N Engl J Med 344: 858-359
  7. Jokar M et al (2015) Epidemiology of Vasculitides in Khorasan Province, Iran. Iran J Med Sci. 40:362-366
  8. Kufner A et al (1985) Allergic granulomatosis (Churg-Strauss syndrome). Dermatologist 36: 274-280
  9. Kowalzick L et al (2011) Disseminated cutaneous involvement in Churg-Strauss syndrome under omalizumab therapy. Act Dermatol 37: 119-120
  10. Masi AT, Hunder GG, Lie JT et al (1990) The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 33: 1094-1100
  11. Noth I et al.(2003) Churg-Strauss syndrome. Lancet 361: 587-94
  12. Prince JE et al (2003) Immunologic lung disease. J Allergy Clin Immunol 111: S613-23
  13. Ratzinger G et al. (2015) The Vasculitis Wheel-an algorithmic approach to cutaneous vasculitis. JDDG 1092-1118
  14. Wieshuber C et al (2011) Erythematous plaques and steroid-induced asthma. JDDG 9:312-313

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Last updated on: 31.12.2020