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Necrotizing Sarcoid Granulomatosis: Possibly Veiled Disease in Endemic Area of Mycobacterial Infection.

Chong Y, Lee EJ, Kang CS, Kim TJ, Song JS, Shim H - J Pathol Transl Med (2015)

View Article: PubMed Central - PubMed

Affiliation: Department of Hospital Pathology, College of Medicine, The Catholic University of Korea, Seoul, Korea.

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Necrotizing sarcoid granulomatosis (NSG) is a rare granulomatous disease that primarily affects the lung and presents as nodular masses of confluent sarcoid-like granulomas with extensive necrosis and vasculitis... In addition to its rarity, the similarity of the clinical, radiological, and pathological features of NSG to other granulomatous diseases, such as granulomatous infection, nodular sarcoidosis, and Wegener’s granulomatosis (WG), is the largest obstacle in its proper diagnosis... The initial symptoms are non-specific or frequently do not present at all... The granulomas were accompanied by central necrosis that was suppurative rather than caseous, palisaded by Langerhans-type giant cells and mononuclear lymphohistiocytes... At first glance, granulomatous vasculitis did not seem evident, but on closer observation, mild transmural vasculitis with fibrosis distant from the necrotic area was clearly noted in a significant portion of the lesion (Fig. 2A–D)... Histological examination of resected tissue revealed variably-sized granulomas with varying amounts of suppurative necrosis and frequent granulomatous vasculitis, raising suspicion for sarcoidosis or other kinds of granulomatous vasculitis (Fig. 2E, F)... However, autoimmune markers were all negative, and no causative microorganisms were found on various tests... First, histologically, NSG appears as sarcoid-like granuloma with vasculitis and necrosis... If one finds a mixture of pathologic features of pulmonary sarcoidosis and mild to moderate granulomatous vasculitis, combined with negative tests for causative microorganisms, the possibility of NSG can be raised... However, conclusive diagnosis cannot be made on histologic features alone, and the final pathologic diagnosis of NSG should be made after thorough exclusion of the other possible diseases with similar features... Most importantly, granulomatous infections should be carefully excluded because they can also produce variable vasculitis, extensive necrosis, and sarcoid-like granulomas... A case of NSG was reported in 1997 by Kim et al., but the evidence for NSG in their report is insufficient because polymerase chain reaction was not used to exclude tuberculosis, the patient was not appropriately treated, and there was no radiographic improvement... In conclusion, the diagnosis of NSG should be made very carefully in suspected cases based on consistent radiologic and histologic findings and thorough exclusion of possible causative microorganisms.

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Related in: MedlinePlus

Microscopic findings. (A–C) Multifocal nodular granulomatous inflammation with extensive central necrosis is replacing normal architecture (case 1). (D) Mild transmural vasculitis distant from necrotic area, i.e., cicatricial-type vasculitis, is observed. (E, F) Multiple confluent granulomas with central caseous necrosis and granulomatous vasculitis are found (case 2).
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f2-jptm-49-4-346: Microscopic findings. (A–C) Multifocal nodular granulomatous inflammation with extensive central necrosis is replacing normal architecture (case 1). (D) Mild transmural vasculitis distant from necrotic area, i.e., cicatricial-type vasculitis, is observed. (E, F) Multiple confluent granulomas with central caseous necrosis and granulomatous vasculitis are found (case 2).

Mentions: On microscopic examination of the wedge-resected tissue, confluent small granulomas with necrosis of variable sizes were seen in the lung parenchyma. The granulomas were accompanied by central necrosis that was suppurative rather than caseous, palisaded by Langerhans-type giant cells and mononuclear lymphohistiocytes. At first glance, granulomatous vasculitis did not seem evident, but on closer observation, mild transmural vasculitis with fibrosis distant from the necrotic area was clearly noted in a significant portion of the lesion (Fig. 2A–D). The differential diagnosis based on pathologic findings included granulomatous diseases of various causes. Special stains for fungus and acid-fast bacilli on the resected tissue and sputum were negative. Tuberculosis–polymerase chain reaction and culture on bronchial washing fluid and peripheral blood were both negative. Serum cryptococcal antigen and IgG for parasites were all negative.


Necrotizing Sarcoid Granulomatosis: Possibly Veiled Disease in Endemic Area of Mycobacterial Infection.

Chong Y, Lee EJ, Kang CS, Kim TJ, Song JS, Shim H - J Pathol Transl Med (2015)

Microscopic findings. (A–C) Multifocal nodular granulomatous inflammation with extensive central necrosis is replacing normal architecture (case 1). (D) Mild transmural vasculitis distant from necrotic area, i.e., cicatricial-type vasculitis, is observed. (E, F) Multiple confluent granulomas with central caseous necrosis and granulomatous vasculitis are found (case 2).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4508575&req=5

f2-jptm-49-4-346: Microscopic findings. (A–C) Multifocal nodular granulomatous inflammation with extensive central necrosis is replacing normal architecture (case 1). (D) Mild transmural vasculitis distant from necrotic area, i.e., cicatricial-type vasculitis, is observed. (E, F) Multiple confluent granulomas with central caseous necrosis and granulomatous vasculitis are found (case 2).
Mentions: On microscopic examination of the wedge-resected tissue, confluent small granulomas with necrosis of variable sizes were seen in the lung parenchyma. The granulomas were accompanied by central necrosis that was suppurative rather than caseous, palisaded by Langerhans-type giant cells and mononuclear lymphohistiocytes. At first glance, granulomatous vasculitis did not seem evident, but on closer observation, mild transmural vasculitis with fibrosis distant from the necrotic area was clearly noted in a significant portion of the lesion (Fig. 2A–D). The differential diagnosis based on pathologic findings included granulomatous diseases of various causes. Special stains for fungus and acid-fast bacilli on the resected tissue and sputum were negative. Tuberculosis–polymerase chain reaction and culture on bronchial washing fluid and peripheral blood were both negative. Serum cryptococcal antigen and IgG for parasites were all negative.

View Article: PubMed Central - PubMed

Affiliation: Department of Hospital Pathology, College of Medicine, The Catholic University of Korea, Seoul, Korea.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Necrotizing sarcoid granulomatosis (NSG) is a rare granulomatous disease that primarily affects the lung and presents as nodular masses of confluent sarcoid-like granulomas with extensive necrosis and vasculitis... In addition to its rarity, the similarity of the clinical, radiological, and pathological features of NSG to other granulomatous diseases, such as granulomatous infection, nodular sarcoidosis, and Wegener’s granulomatosis (WG), is the largest obstacle in its proper diagnosis... The initial symptoms are non-specific or frequently do not present at all... The granulomas were accompanied by central necrosis that was suppurative rather than caseous, palisaded by Langerhans-type giant cells and mononuclear lymphohistiocytes... At first glance, granulomatous vasculitis did not seem evident, but on closer observation, mild transmural vasculitis with fibrosis distant from the necrotic area was clearly noted in a significant portion of the lesion (Fig. 2A–D)... Histological examination of resected tissue revealed variably-sized granulomas with varying amounts of suppurative necrosis and frequent granulomatous vasculitis, raising suspicion for sarcoidosis or other kinds of granulomatous vasculitis (Fig. 2E, F)... However, autoimmune markers were all negative, and no causative microorganisms were found on various tests... First, histologically, NSG appears as sarcoid-like granuloma with vasculitis and necrosis... If one finds a mixture of pathologic features of pulmonary sarcoidosis and mild to moderate granulomatous vasculitis, combined with negative tests for causative microorganisms, the possibility of NSG can be raised... However, conclusive diagnosis cannot be made on histologic features alone, and the final pathologic diagnosis of NSG should be made after thorough exclusion of the other possible diseases with similar features... Most importantly, granulomatous infections should be carefully excluded because they can also produce variable vasculitis, extensive necrosis, and sarcoid-like granulomas... A case of NSG was reported in 1997 by Kim et al., but the evidence for NSG in their report is insufficient because polymerase chain reaction was not used to exclude tuberculosis, the patient was not appropriately treated, and there was no radiographic improvement... In conclusion, the diagnosis of NSG should be made very carefully in suspected cases based on consistent radiologic and histologic findings and thorough exclusion of possible causative microorganisms.

No MeSH data available.


Related in: MedlinePlus