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A case of digital vasculitis

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Q1: What is the most probable diagnosis?

The diagnosis is Churg-Strauss syndrome on the basis of digital vasculitis, Raynaud's phenomenon, mild peripheral neuropathy, mononeuritis multiplex, marked eosinophilia, and a past history of asthma.

Q2: What is the treatment?

The treatment is steroids. Patients with poor prognostic features, such as myocardial and gastrointestinal involvement, can be treated with combined steroids and cyclophosphamide.

Discussion

Churg-Strauss syndrome (CSS) is a granulomatous vasculitis affecting multiple organ systems. It may resemble polyarteritis nodosa except for the high degree of lung involvement, vasculitis of mainly small sized vessels, extravascular granuloma formation, eosinophilic tissue infiltration, peripheral eosinophilia, and a strong association with severe asthma.1

The American College of Rheumatology has proposed six criteria for the diagnosis of CSS. Four of the six criteria are necessary for a diagnosis with 85% sensitivity and 99.7% specificity. These criteria include a history of asthma, the presence of eosinophilia >1500/mm3, paranasal sinusitis, pulmonary infiltrates seen on radiography, histological proof of vasculitis, and mononeuritis multiplex.2

Asthma is the most frequently observed presentation but may precede the development of systemic vasculitis by up to 30 years.3Mononeuritis multiplex is the second commonest manifestation. Other common features are fever, malaise, weight loss, fleeting pulmonary infiltrates, arthralgia, and gastrointestinal involvement. Cardiac involvement is seen in one third of patients, with myocardial and endocardial involvement being common. Skin lesions are seen in 70% of cases, usually in the form of purpura, cutaneous and subcutaenous nodules. Glomerular involvement is rare. Gastrointestinal tract involvement is due to mesenteric vasculitis and usually presents with abdominal pain.3 4

Although CSS may be readily diagnosed on clinical grounds, histological confirmation should always be sought. The classical picture consists of necrotising vasculitis, eosinophilic tissue infiltration, and extravascular granulomas, but it is only found in a minority of patients and is not pathognomic of CSS.5

Antineutrophil cytoplasmic antibodies, especially antimyeloperoxidase, are positive in 60%–75% of patients with CSS.5

The prognosis is poor with 25% five year survival if untreated. Treatment with steroids dramatically increases the chance of survival. Patients with poor prognostic features, such as myocardial and gastrointestinal involvement, should be treated with combined steroids and cyclophosphamide.

Our patient had four out of the six American College of Rheumatology criteria, namely asthma, eosinophilia, pulmonary infiltrates, and mononeuritis multiplex. Her cardiac involvement warranted the use of cyclophosphamide in addition to methylprednisolone.

Final diagnosis

Churg-Strauss syndrome.

References

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