An unusual presentation of Wegener’s granulomatosis with Splenic infarct
P. Radhakrishnan*a (Dr), Y. Sivagnanamea (Prof), A. Selvama (Dr)
a Sri Manakula Vinayagar Medical College and Hospital, Puducherry, INDIA
Granulomatosis with polyangiitis (GPA) or Wegener’s granulomatosis is a necrotizing vasculitides predominantly affecting small-sized arteries. It is a rare long term systemic disorder that affects most commonly the upper and lower respiratory tract and the kidneys.
A 21-year-old male, driver by occupation presented with complaints of breathlessness, cough with blood stained sputum and high-grade fever for 15 days. He is non-smoker and non-alcoholic with no comorbidities. His vitals were stable with oxygen saturation of 95% @ room air. Routine blood investigation showed neutrophilic leukocytosis and thrombocytosis. Blood culture showed non-fermenting gram negative bacilli and was started on antibiotics as per the sensitivity along with supportive measures. Chest radiography showed left upper zone mass. Computed Tomography thorax showed bilateral multiple nodular masses and few of them showing cavities with diffuse ground glass opacities. Patient complained of abdominal pain on fifth day of admission. Initially he was evaluated with bedside Ultrasonogram which showed heterogenous spleen with perisplenic collection. Later, contrast enhanced CT abdomen was done which showed non-enhancing spleen suggestive of splenic infarct. Echocardiography was suggestive of minimal pericardial effusion. Otolaryngology and ophthalmologic evaluation were normal. Diagnostic bronchoscopy showed pale, unhealth mucosa with right and left upper lobe bronchus narrowing. BAL Cytology showed neutrophilic predominance. BAL AFB, culture and CBNAAT were negative. Endobronchial biopsy was suggestive of granulomatous inflammation. With suspicion of GPA, ANCA profile was sent. C -ANCA was strongly positive (345.01 RU/mL). ANA was negative. Patient was started on methylprednisolone pulse therapy along with Rituximab. Patient improved symptomatically with significant radiological resolution and is on follow up.
GPA is a systemic disease that can lead to multiorgan failure when remained untreated. We encountered a rare unusual presentation of GPA with splenic infarct with normal renal function and absence of ENT and eye involvement.
Disclosure of funding source(s): none