Common variable immunodeficiency (CVID) is the most prevalent symptomatic type of human primary immunodeficiency disease (PID). Clinically, CVID is characterized by increased susceptibility to infections and a wide variety of automimmune and rheumatologic disorders. The most common rheumatologic manifestations are juvenile idiopathic arthritis (JIA) and adult rheumatoid arthritis (RA). The frequency of rheumatologic diseases in patients with CVID is 5-13%. The most prevalent rheumatologic diseases seen in patients with CVID are RA (2-3%), JIA (1-13%), Sjogren's syndrome (1-8%) and SLE (1%). We report a rare case of JIA in patient with CVID.
Case Report : 7-year old male visited pediatric rheumatology clinic. He complaint recurrent otitis media and sinusitis since 4-year old age. Recently he developed Rt wrist joint swollen with pain.
Tc-99m HDP WBBS shows : increased bony uptake in Rt carpal bones, both 2nd 3rd MCP and 3rd PIP joints, suggesting arthritis. UltraSonography : synovial thickening with increased vascularity of the Rt carpometacarpal, intercarpal and radiocarpal joint without visible joint effusion, JIA most likely. CBC 6,900/mm3 Hb 11.9 g/dL platelet 423k (neutrophil 2% lymphocyte 74% monocyte 22%) ESR 14mm/hr. CRP 12.5 mg/L. IgG <50 mg/dL IgA <10 mg/dL IgM <10 mg/dL IgE <10 mg/dL. C3/C4 134/69. CH50 195. T cell (CD3) 98% Th cell (CD4) 63.5% Ts cell (CD8) 33.9%. Th/Ts ratio 1.87. B cell (CD19) 0.0 %. NK cell 1.9 %.
Rheumatologic complications can be the presenting symptom of immunodeficiency prior to the definite diagnosis of CVID. Particularly when accompanied by infectious and autoimmune complications.