Case Report
Necrotizing Sarcoid Granulomatosis: A Disease Not to be Forgotten
Table 2
Characteristics of classical variant (nodular sarcoidosis) and necrotizing variant (NGS) [
2,
5].
| | | Nodular sarcoidosis | Necrotizing variant |
| | Epidemiology | Prevalence: 10 to 20 per 100,000 population
Males 44%
Females 56%
Median age: 35 | <300 cases have been reported
Males 37%
Females 63%
Median age: 42 | | Histology | Nonnecrotizing epithelioid granulomas | Granulomas
Necrosis (coagulative or caseous) and vasculitis
Foci of infarction | | Clinical presentation | 88%
Pulmonary and/or systemic symptoms (fever, weight loss, night sweats, malaise, and so on) | 84%
Pulmonary and/or systemic symptons (fever, weight loss, night sweats, malaise, and so on) | | Involved organs | | | | >>SACE elevation | 17% | 4% | | >>Eye involvement | 14% | 12% | | >>Skin involvement | 10% | 2% | | >>Lymphadenopathy | 9% | 0.5% | | >>Liver involvement | 9% | 1% | | >>Erithema nodosum | 3% | 1% | | >>Sjögren or sicca syndrome | 1% | 3% | | >>CNS involvement | 2% | 7% | | >>Neuropathy | 0% | 2% | | >>Splenic involvement | 2% | 1% | | >>Lacrimal gland involvement | 1% | 2% | | Diagnosis | Transbronchial lung biopsy (35%)
Tissue obtained by surgical procedures (33%)
Needle biopsy (9%)
Bronchial biopsy (2%)
Intrathoracic lymph node biopsy (8%)
Extrathoracic lymph node biopsy (3%) | Tissue obtained by surgical procedures (98%) |
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SACE: serum angiotensin converting enzyme; CNS: central nervous system.
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