An aggressive and lethal course of Churg–Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy

Çiledağ, Aydın; Deniz, Hülya; Eledağ, Serpil; Özkal, Canan; Düzgün, Nurşen; Erekul, Selim; Karnak, Demet

doi:10.1007/s00296-009-1321-9

Cited by 15 publications

(16 citation statements)

References 17 publications

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“…Pleural effusion, secondary to heart failure or to direct eosinophilic involvement, is observed in 12% of cases [38]. Although extremely rare, alveolar hemorrhage has been reported in EGPA [42][43][44][45][46][47].…”

Section: Eosinophilic Granulomatosis With Polyangiitismentioning

confidence: 99%

The Respiratory System in Autoimmune Vascular Diseases

Nasser¹,

Cottin²

2018

Respiration

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The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis, affects the respiratory tract more commonly than other vasculitis types. Granulomatosis with polyangiitis is always associated with upper or lower respiratory tract involvement. Pulmonary and ENT involvements are the hallmark feature of the disease and are present in 90 and 80% of cases, respectively, with frequent skin or gastrointestinal involvement. In about 10% of cases, the lung is the only organ affected. Eosinophilic granulomatosis with polyangiitis is always associated with hypereosinophilia and asthma which usually precedes the systemic manifestations by several years; however, onset of asthma and of the vasculitis may be concomitant. Parenchymal infiltrates may be migratory and rapidly resolve upon corticosteroid treatment. Diffuse alveolar hemorrhage and renal failure are typical features of microscopic polyangiitis. The former is the leading manifestation of anti-glomerular basement membrane disease and is usually part of a pulmonary-renal syndrome. Takayasu arteritis has a distinct clinical presentation due to pulmonary arteritis and may present with massive hemoptysis, chest pain, and rarely symptoms of pulmonary hypertension. Behçet disease is the most common cause of pulmonary artery aneurysm and can also cause in situ thrombosis of the pulmonary arteries. Corticosteroids and immunosuppressive agents are the mainstay of treatment. In conclusion, systemic vasculitis is a frequent cause of respiratory system involvement with diverse manifestations of distinct severity and outcome.

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Section: Eosinophilic Granulomatosis With Polyangiitismentioning

confidence: 99%

The Respiratory System in Autoimmune Vascular Diseases

Nasser¹,

Cottin²

2018

Respiration

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“…Eosinophilic granulomatosis with polyangiitis (EGPA) belongs to the systemic vasculitides which may develop life-threatening major organ involvement Any type of vasculitis has the potential to cause local or diffuse pathological changes in the gastrointestinal (GI) tract based on the inflammation of vessel walls, followed by alterations of the blood flow and ischemic damage to the dependent organ (Çileda» et al 2012; Singh et al 2009; Venditti et al 2011). Intestinal manifestations have to be considered more common in other vasculitis disease, such as polyarteriitis nodosa, however, it seems to occur as severe manifestation of EGPA (Nakamura et al 2002).…”

Section: Introductionmentioning

confidence: 99%

Gastrointestinal perforation due to vasculitis at primary diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) despite a high dose glucocorticosteroids treatment

et al. 2014

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BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) belongs to the systemic ANCA-associated vasculitides which may develop life-threatening major organ involvement, such as eosinophilic pulmonary infiltration, neuropathy, acute nephritis, myocarditis, and gastrointestinal (GI) tract involvement. Here, two cases of EGPA are presented developing perforation of the bowel at primary diagnosis after 7 respectively 10 days of initiation of high-dose glucocorticosteroides (GC) therapy.FindingsTwo male patients, aged 32 (case 1) and 36 years (case 2), were admitted to the hospital with the symptoms of dyspnea, fatigue, fever, and chest pain. The patients completed the previously published revised international Chapel Hill nomenclature of EGPA. The two patients (case 1 and 2) developed acute abdominal pain after 7 (case 1) and ten days (case 2) on GC treatment. Computed tomography followed by surgery detected acute perforation of the small intestine (middle part of jejunum, case 1) and colon transversum (case 2). The resected specimens disclosed bowel perforation with severe transmural inflammation, edema, hemorrhage and vasculitis typically of EGPA. On the first post-operative day, therapy with cyclophosphamide (according to the Austin protocol) with a dosage of 750 mg/qm every 3 weeks (for 8 cycles) was initiated with good response within three months of treatment.ConclusionThe course of disease of the two presented EGPA patients suggest that very early initiation of intensified immunosuppressive treatment, preferentially with cyclophophomide or B cell depletion strategies, needs to be considered to avoid life-threatening complications of GI involvement.

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“…Ulceration, perforation, and stenosis of the gastrointestinal tract are assumed to be the results of ischemia caused by vasculitis. The small intestine is the most commonly affected site[ 8 ]. Immunosuppressive therapy, especially large-dosage corticosteroids, may play an important role in the development of an intestinal perforation.…”

Section: Dicussionmentioning

confidence: 99%

Multiple perforations and fistula formation following corticosteroid administration: A case report

He¹,

Tian²,

Xu³

et al. 2017

WJCC

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Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic small- and medium-sized-vessel vasculitis. The literature contains only a few reports of gastrointestinal perforation with this condition. We report a patient with EPGA treated with high-dose steroid who underwent emergency surgery for intestinal perforations. We performed a simple repair of the 11 perforations. Intestinal fistulas developed 8 d postoperatively; they healed well after 60 d of continuous washing and negative pressure suction. The clinical data of 14 additional patients with EGPA or Churg-Strauss syndrome complicated with gastrointestinal perforation, which were reported from 1996 to 2014, were also collected and compared. The formation of multiple perforations and fistulas following high dosage steroid administration can have a good outcome with appropriate management. Meticulous attention to abdominal symptoms and appropriate interventions can result in timely management. Corticosteroid administration remains a very important perioperative procedure for EPGA.

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An aggressive and lethal course of Churg–Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy

Cited by 15 publications

References 17 publications

The Respiratory System in Autoimmune Vascular Diseases

The Respiratory System in Autoimmune Vascular Diseases

Gastrointestinal perforation due to vasculitis at primary diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) despite a high dose glucocorticosteroids treatment

Multiple perforations and fistula formation following corticosteroid administration: A case report

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