A 40-year-old male, mining sounder.
She had a history of erythema nodosum of unknown etiology in 1995 and positive serology for Chagas disease in 2011, performed as a result of radiological cardiomegaly.
Since February 2013, the patient presented with edema of the lower limbs that progressed with compromise and compromise.
The echocardiographic evaluation detected an extensive pericardial effusion without signs of tamponade, performing a pericardiocentesis with extraction of 840 cc of citrine liquid, with negative microbiological analysis.
Due to the rapid reproduction of the effusion, a pericranial window was performed.
The pericardial tissue biopsy showed a predominantly lymphoplasmacytic infiltrate and foci of microarrhagia.
Immunohistochemical analysis determined the polyclonality of the infiltrate.
Computed tomography of the neck, chest, abdomen and pelvis revealed pansinusitis, multiple mediastinal lymphadenopathy, pulmonary sequels and retroperitoneal wall thickening, pericardial effusion suggestive of chronic ascending pericarditis, left pleural tuberculosis.
Among laboratory tests, hemoglobin 12.2 g/dL, platelets and normal leukocyte counts and formula, ESR 46 mm/h stood out.
Renal and hepatic function, urine test and biochemical profile showed no abnormalities.
Lactate dehydrogenase (LDH) was 131 IU/L (normal 230-460), albumin 3.3 g/dL (normal 3.5-5.2), and β2 microglobulin 0.8-2.35 mg/L.2).
The viral study was negative for HIV, HBV, HCV and HTLV-1.
The rheumatoid arthritis study was negative for antinuclear antibodies and rheumatic factor.
The immunoglobulin count was IgA 231 mg/dL (normal 70-400), IgG 1316 mg/dL (normal 700-1,600) and IgM 23 mg/dL (normal 40-230).
In relation to urethral injury of the right lung apex, she had a 27 mm PPD, with positive Quantiferon and negative bacilloscopy.
Because of the lack of an etiologic diagnosis, a mediastinal lymph node biopsy was performed and reported as ganglionic cystocytosis.
Due to the presence of polyadenopathies, pericardial and pleural effusion, polyclonal lymphoplasmacytic infiltrate was identified and IgG subclass/dL was determined (Evidence of IgG4 elevation in 156 mg-121).
Immunohistochemical analysis for IgG4 ganglion tissue was positive, with a 40% IgG4/IgG plasma cell ratio.
Finally, Koch culture of the same tissue was negative.
1.
A disease related to IgG4 with lymph node, pericardial, pleural, aortic involvement and latent tuberculosis was diagnosed, starting therapy with prednisone 40 mg/day associated with isoniazid 300 mg/day.
One month after treatment, the imaging control showed a dramatic regression of the pericardial and pleural effusion, as well as of the lymphadenopathies described.
As for the catheter, improvement was less noticeable, with partial reduction of thickening of the artery wall.
