Necrotizing Sarcoid Granulomatosis is a newly described atypical form of granulomatous lung diseases with only a few cases described in pulmonary literature especially with extra pulmonary involvement.
We present a case of 25 yrs old Caucasian female, who was referred to pulmonary clinic due to ‘non resolving pneumonia’. She had been developing non-productive cough, fevers and chills for few days. Also, the patient had significant weight loss of around 15 lbs over 2 months. She denied any hemoptysis or pleuritic chest pain. She denied any sick contact and all her past PPD skin tests had been negative. She is a lifetime nonsmoker. Her occupational history was positive for being a neuroscience major and had worked with bovine pituitary glands in the past.Her initial imaging revealed multiple moderate sized nodules distributed between her right and left lungs with no significant hilar involvement. The patient underwent bronchoscopy with transbronchial biopsy, which revealed extensive necrotizing granulomatous inflammation. Also her right lacrimal gland was to swollen and on biopsy was found to be granuloma with giant cell formation. The patient underwent extensive workup including special staining of her BAL, AFB, PCR for mycobateria, fungal serology, connective tissue diseases, febrile agglutinin panel, and brucella antibodies which was all negative. Looking at the systemic illness and weight loss, initially the patient was started on empiric treatment with itraconazole, anti tubercular treatment and steroids. Her symptoms and the chest imaging improved significantly. She was taken off from antifungal and antitubercular medicines slowly as there was no evidence of infections and she continued to improve on steroids. Later on her steroids were also tapered off slowly. Unfortunately, the patient had relapse of her symptoms and reappearance of her pulmonary nodules, which again resolved with reintroduction of steroids and then steroids sparing agents. Over the course of past 3 years, whenever she comes off steroids or immunosuppressive therapy, she has recurrence of her pulmonary nodules with subsequent improvement on steroids.
Necrotizing Sarcoid Granulomatosis (NSG) is a relatively rare disease. Liebow first described it in 1973. In his publication he also defined 4 other forms of pulmonary angitis and granulomatosis: Wegener’s Granulomatosis, Limited Wegener’s Disease, Lymphomatoid Granulomatosis, and Bronchocentric Granulomatosis. Since then, several case reports have documented the existence of NSG although its relationship to classical sarcoidosis remains a matter of debate. NSG differs from sarcoidosis by the additional presence of fibrinoid necrosis. It is estimated that between 1.6 –4% of Pulmonary sarcoidosis can present as the NSG variant. Almost all cases of NSG described are limited to the lung and to date only a handful of cases have been described with extra pulmonary involvement. When it does occur in the extra pulmonary form it seems to have a predilection mainly for the eyes and CNS. In fact, two previously reported cases seemed to indicate that the eye involvement might predate the pulmonary involvement by about 10 –12 years. NSG has been shown to follow a relatively benign course in almost all cases reported with only a few presenting as initial critical illness, but they all seem to respond well to steroids.
As mentioned above, our case represents one of the very few reported cases of Necrotizing Sarcoid Granulomatosis with extra pulmonary involvement. This entity should also be kept in mind as differential diagnosis of necrotizing granulomatous diseases.
Rajan Goyal, No Financial Disclosure Information; No Product/Research Disclosure Information