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Rare manifestations of sarcoidosis in modern era of new diagnostic tools.

Sharma SK, Soneja M, Sharma A, Sharma MC, Hari S - Indian J. Med. Res. (2012)

Bottom Line: Fourteen patients had airways obstruction and behaved typically like seasonal bronchial asthma with excellent response to corticosteroids.Increased awareness of rare manifestations will facilitate better management of these patients.With increasing use of modern diagnostic tools, manifestations hitherto considered rare, are likely to be recognized more frequently in the future.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, All India Institute of Medical Sciences, New Delhi, India. sksharma.aiims@gmail.com

ABSTRACT

Background & objectives: Growing body of literature on sarcoidosis in India has led to an increased awareness of the disease. With the advent of better imaging tools hitherto under-recognized manifestations of sarcoidosis are likely to be better recognized. We sought to study the rare clinical and radiological manifestations (<5%) in patients with sarcoidosis.

Methods: Retrospective review of records of 164 patients with histopathologically proven sarcoidosis seen over six years in a tertiary care centre in north India, was done.

Results: Fifty four rare manifestations were observed in 164 patients. Acute presentation in the form of Lofgren syndrome was seen in eight (4.9%) and Heerfordt's syndrome in two (1.2%) patients. Musculoskeletal manifestations included chronic sarcoid arthritis in three (1.8%), deforming arthritis and bone erosion in one (0.6%) each. Rare initial presentation with dilated cardiomyopathy in one (0.6%), complete heart block in two (1.2%), bilateral sequential facial nerve palsy in two (1.2%), and pyrexia of unknown origin was seen in one (0.6%) patient. Other rare manifestations included chronic respiratory failure in one (0.6%), dysphagia in one (0.6%), sicca syndrome in five (3%), massive splenomegaly in one (0.6%), portal hypertension in two (1.2%), hypersplenism, gastric sarcoidosis, ninth and tenth cranial nerve palsies, moderate pericardial effusion and nephrocalcinosis in one (0.6%) each, and pulmonary artery hypertension in two (1.2%) patients. Rare radiological manifestations included moderate pleural effusion in two (1.2%), pleural thickening in five (3%), calcification of intrathoracic lymph nodes in four (2.4%), alveolar (nodular) sarcoidosis in three (1.8%), and myocardial uptake of 18F-fluorodeoxyglucose (F-18 FDG) in two (1.2%) patients. Fourteen patients had airways obstruction and behaved typically like seasonal bronchial asthma with excellent response to corticosteroids.

Interpretation & conclusions: Increased awareness of rare manifestations will facilitate better management of these patients. With increasing use of modern diagnostic tools, manifestations hitherto considered rare, are likely to be recognized more frequently in the future.

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Related in: MedlinePlus

Histopathological images. (A) Cut surface of spleen showing nodular lesions. (B) Microscopic examination from the lesions showing necrotizing granulomatous reaction with Langhans giant cells (arrows) on H&E stain; 400×. (C) These granulomas are rich in reticulin fibers as seen on silver stain; 200×. (D & E) Liver biopsy showing granulomatous reaction in the portal tract with Langhans giant cells and areas of necrosis (arrow in D and arrow head in E) on H&E stain at 100 & 200x respectively. (F) Arthroscopic synovial biopsy showing non-caseating granulomas (arrow) on H&E stain; 100×.
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Figure 5: Histopathological images. (A) Cut surface of spleen showing nodular lesions. (B) Microscopic examination from the lesions showing necrotizing granulomatous reaction with Langhans giant cells (arrows) on H&E stain; 400×. (C) These granulomas are rich in reticulin fibers as seen on silver stain; 200×. (D & E) Liver biopsy showing granulomatous reaction in the portal tract with Langhans giant cells and areas of necrosis (arrow in D and arrow head in E) on H&E stain at 100 & 200x respectively. (F) Arthroscopic synovial biopsy showing non-caseating granulomas (arrow) on H&E stain; 100×.

Mentions: One patient died during the study period due to cardiac complications. One patient developed chronic respiratory failure and is on domiciliary oxygen therapy. The remaining patients with rare manifestations had good response to treatment. Selected clinical, radiological and histopathology images are shown in Figs 3,4 and 5, respectively.


Rare manifestations of sarcoidosis in modern era of new diagnostic tools.

Sharma SK, Soneja M, Sharma A, Sharma MC, Hari S - Indian J. Med. Res. (2012)

Histopathological images. (A) Cut surface of spleen showing nodular lesions. (B) Microscopic examination from the lesions showing necrotizing granulomatous reaction with Langhans giant cells (arrows) on H&E stain; 400×. (C) These granulomas are rich in reticulin fibers as seen on silver stain; 200×. (D & E) Liver biopsy showing granulomatous reaction in the portal tract with Langhans giant cells and areas of necrosis (arrow in D and arrow head in E) on H&E stain at 100 & 200x respectively. (F) Arthroscopic synovial biopsy showing non-caseating granulomas (arrow) on H&E stain; 100×.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3401691&req=5

Figure 5: Histopathological images. (A) Cut surface of spleen showing nodular lesions. (B) Microscopic examination from the lesions showing necrotizing granulomatous reaction with Langhans giant cells (arrows) on H&E stain; 400×. (C) These granulomas are rich in reticulin fibers as seen on silver stain; 200×. (D & E) Liver biopsy showing granulomatous reaction in the portal tract with Langhans giant cells and areas of necrosis (arrow in D and arrow head in E) on H&E stain at 100 & 200x respectively. (F) Arthroscopic synovial biopsy showing non-caseating granulomas (arrow) on H&E stain; 100×.
Mentions: One patient died during the study period due to cardiac complications. One patient developed chronic respiratory failure and is on domiciliary oxygen therapy. The remaining patients with rare manifestations had good response to treatment. Selected clinical, radiological and histopathology images are shown in Figs 3,4 and 5, respectively.

Bottom Line: Fourteen patients had airways obstruction and behaved typically like seasonal bronchial asthma with excellent response to corticosteroids.Increased awareness of rare manifestations will facilitate better management of these patients.With increasing use of modern diagnostic tools, manifestations hitherto considered rare, are likely to be recognized more frequently in the future.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, All India Institute of Medical Sciences, New Delhi, India. sksharma.aiims@gmail.com

ABSTRACT

Background & objectives: Growing body of literature on sarcoidosis in India has led to an increased awareness of the disease. With the advent of better imaging tools hitherto under-recognized manifestations of sarcoidosis are likely to be better recognized. We sought to study the rare clinical and radiological manifestations (<5%) in patients with sarcoidosis.

Methods: Retrospective review of records of 164 patients with histopathologically proven sarcoidosis seen over six years in a tertiary care centre in north India, was done.

Results: Fifty four rare manifestations were observed in 164 patients. Acute presentation in the form of Lofgren syndrome was seen in eight (4.9%) and Heerfordt's syndrome in two (1.2%) patients. Musculoskeletal manifestations included chronic sarcoid arthritis in three (1.8%), deforming arthritis and bone erosion in one (0.6%) each. Rare initial presentation with dilated cardiomyopathy in one (0.6%), complete heart block in two (1.2%), bilateral sequential facial nerve palsy in two (1.2%), and pyrexia of unknown origin was seen in one (0.6%) patient. Other rare manifestations included chronic respiratory failure in one (0.6%), dysphagia in one (0.6%), sicca syndrome in five (3%), massive splenomegaly in one (0.6%), portal hypertension in two (1.2%), hypersplenism, gastric sarcoidosis, ninth and tenth cranial nerve palsies, moderate pericardial effusion and nephrocalcinosis in one (0.6%) each, and pulmonary artery hypertension in two (1.2%) patients. Rare radiological manifestations included moderate pleural effusion in two (1.2%), pleural thickening in five (3%), calcification of intrathoracic lymph nodes in four (2.4%), alveolar (nodular) sarcoidosis in three (1.8%), and myocardial uptake of 18F-fluorodeoxyglucose (F-18 FDG) in two (1.2%) patients. Fourteen patients had airways obstruction and behaved typically like seasonal bronchial asthma with excellent response to corticosteroids.

Interpretation & conclusions: Increased awareness of rare manifestations will facilitate better management of these patients. With increasing use of modern diagnostic tools, manifestations hitherto considered rare, are likely to be recognized more frequently in the future.

Show MeSH
Related in: MedlinePlus