Granulomatous disease in common variable immunodeficiency

Abstract

Granulomatous disease occurs in 8-22% of patients with common variable immunodeficiency (CVID). We examined the clinical and immunologic information of all 37 of 455 (8.1%) CVID subjects with this complication. The median age at diagnosis of CVID was 26 (2-59). 14 had granulomas 1-18 years before diagnosis of CVID. In 6 detection of granulomas coincided with this diagnosis; for 17, granulomas were documented later. 54% had lung granulomas, 43% in lymph nodes and 32% in liver. 54% of the group had had autoimmune diseases, mostly immune thrombocytopenia and hemolytic anemia. 24% had had a splenectomy. Nineteen (51.3%) required steroid treatment for granulomas; other immune suppressants were used in some. Over 25 years 28.5% died (median age 37.5), but not significantly more when compared to our CVID patients without granulomas (19.8%). Those with lung granulomas had similar mortality to those with granulomas in other tissues.

Figure 1

The abdominal computed tomographic scan of subject # 15 who had a complex and mixed attenuation mass within the small bowel mesentery. This involved the spleen and surrounding tissues to the left of the midline. The arrow marks the left upper quadrant where the adhesive tissues in this area proved to be a collection of large granulomata. A splenectomy was performed.

Figure 2

Shows the spleen of subject # 19. She had a history of intractable AIHA and the splenectomy was done for this reason. The surface of the spleen revealed multiple white surface and interior lesions that were shown to be granulomas on histologic stains.

Figure 3

Shows a histologic H&E stain revealing a solitary granuloma in the bone marrow of Subject # 26, done because she had ongoing ITP.