All Consolidations are not Infective: Look Beyond ARDS.

Dear Editor,

Wegener's granulomatosis (WG) is an uncommon multisystem disease of autoimmune etiology. A 40-year-old female presented to the emergency department (ED) with complaints of worsening fever, productive cough, and difficulty in breathing over 1 month. The patient had an oxygen saturation of 85% with a respiratory rate of 36 bpm and heart rate of 120 bpm. She had bilateral fine crepitations heard all over the chest. Her arterial blood gases was suggestive of type I respiratory failure. Chest X-ray revealed bilateral diffuse reticulonodular opacities [Figure 1]. The blood tests were unremarkable except for an elevated erythrocyte sedimentation rate of 65 mm in 1 h.

Figure 1

Computed tomography scan showing bilateral consolidation with scattered centrilobular nodules

The patient was intubated in view of impending respiratory failure. Empirical antibiotic therapy was initiated for bilateral lung consolidations. Computed tomography (CT) chest was done which showed bilateral extensive consolidation with multiple scattered centrilobular nodules [Figure 2]. Urine examination revealed proteinuria which prompted for further evaluation with autoimmune workup, in which cytoplasmic antineutrophil cytoplasmic antibody (C-ANCA) was found to be positive. Immediate CT-guided biopsy was performed which reported the lesion as suggestive of WG.

Figure 2

Chest X-ray showing bilateral reticulonodular opacities

WG is a rare and invariably fatal form of systemic vasculitis, but early diagnosis and management can have significant impact on disease outcome. Lung nodules are the most common manifestation of WG and occur in approximately 40%–70% of patients.[1] WG nodules may occur in a centrilobular distribution, mimicking tuberculosis, hypersensitivity pneumonitis, or even an acute viral, bacterial, or fungal pneumonia making a pretty huge list of differentials.[2] C-ANCA has a high degree of association with WG but may rarely be elevated in other autoimmune diseases such as microscopic polyangiitis, ChurgStrauss syndrome, systemic lupus erythematosus, polyarteritis nodosa, and Takayasu disease.[3] Lung consolidation and ground-glass opacity often occur in approximately 30% of patients with active WG and are usually the result of hemorrhage.[4] This disease also involves skin, eyes, nervous system, and joints at some stage of the disease course.[5] With current treatment, mortality has improved, but morbidity remains considerably high. According to a meta-analysis, the 5-year survival rate ranges from 74%–79% with current treatment.[6] Treatment includes immunosuppressant therapy, most commonly systemic steroids and cyclophosphamide. Remission rates are approximately 90%, but relapses may occur. Resolution of imaging findings of WG may lag behind clinical improvement. This case emphasizes on the high degree of suspicion needed in the ED where all consolidations are likely to be taken as infective and managed on similar lines delaying the diagnosis of this potentially lethal condition, leading to poor outcome.

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