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Enthesitis as a component of dactylitis in psoriatic juvenile idiopathic arthritis: histology of an established clinical entity.

Tuttle KS, Vargas SO, Callahan MJ, Bae DS, Nigrovic PA - Pediatr Rheumatol Online J (2015)

Bottom Line: Fibrinous synovitis and mild reactive synoviocyte hyperplasia were seen.Histologic hallmarks include an expanded mast cell-populated extracellular myxoid matrix, cartilage degeneration, and a T cell-rich perivascular inflammatory infiltrate.These findings help to define enthesitis as a clinicopathologic entity.

View Article: PubMed Central - PubMed

Affiliation: Division of Immunology, Boston Children's Hospital, Boston, MA USA.

ABSTRACT

Context: Imaging of dactylitis in adult psoriatic arthritis suggests a pathophysiological role for enthesitis. However, histological definition of the dactylitic digit is unavailable.

Objective: We evaluated the role of enthesitis in dactylitis associated with psoriatic juvenile idiopathic arthritis (psJIA) in a child who underwent detailed imaging and histologic evaluation.

Design: Radiographs, ultrasound and high-resolution magnetic resonance imaging were employed to define the locus of disease in two dactylitic digits from a 14-year-old girl with psJIA. Biopsies were stained with hematoxylin and eosin, CD3, CD20, CD4, CD8 and CD117 and examined via light microscopy.

Results: Radiologic features of dactylitis included enhanced signal at digital entheses without accompanying synovitis or tenosynovitis. Histologically, finger and toe tissue exhibited hypervascular tenosynovium with a fibromyxoid expansion of fibrous tissue. This was accompanied by sparse to moderate perivascular lymphocytic inflammation consisting predominantly of T cells, with occasional admixed B cells and mast cells. Neutrophils and plasma cells were absent. Fibrocartilage exhibited reactive features including increased extracellular myxoid matrix, binucleation, and focal necrosis, without cellular inflammation. Fibrinous synovitis and mild reactive synoviocyte hyperplasia were seen.

Conclusions: Dactylitis in psJIA bears a radiographic resemblance to the condition in adult psoriatic arthritis. Histologic hallmarks include an expanded mast cell-populated extracellular myxoid matrix, cartilage degeneration, and a T cell-rich perivascular inflammatory infiltrate. These findings help to define enthesitis as a clinicopathologic entity.

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Related in: MedlinePlus

Characterization of cellular inflammation. Hypervascular tenosynovium showing perivascular inflammation consisting predominantly of T cells (finger; A, hematoxylin and eosin; B, CD3 immunostain; original magnification, 400×). In areas where myxoid matrix expands fibrovascular tissue containing densely collagenized tendon-like fibers, mast cells are regularly interspersed (finger; C, hematoxylin and eosin; D, CD117 immunostain; original magnification, 400×).
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Fig5: Characterization of cellular inflammation. Hypervascular tenosynovium showing perivascular inflammation consisting predominantly of T cells (finger; A, hematoxylin and eosin; B, CD3 immunostain; original magnification, 400×). In areas where myxoid matrix expands fibrovascular tissue containing densely collagenized tendon-like fibers, mast cells are regularly interspersed (finger; C, hematoxylin and eosin; D, CD117 immunostain; original magnification, 400×).

Mentions: Clinical concern persisted regarding typical or atypical mycobacteria as a cause of the patient’s dactylitis, particularly in view of recent treatment with cyclophosphamide and planned tumor necrosis factor (TNF) blockade. Accordingly, deep soft tissue biopsies were performed on both swollen digits. Histologic examination of both the toe and finger showed hypervascular tenosynovium (Figure 4). Cellular inflammation (Figure 5) was mild in the toe and moderate in the finger, consisting predominantly of CD3-positive, CD5-positive, and CD4-positive T cells in both specimens, with rare CD8-positive cells. Sparse interspersed CD20-positive B cells and CD117-positive mast cells were also seen. The toe showed a small component of fibrocartilage that lacked cellular inflammation but showed reactive features including excess myxoid extracellular matrix and occasional binucleate and multinucleate chondrocytes. The finger revealed fibrinous exudate along a synovial surface and focal hypocellularity and coagulative necrosis of fibrocartilage. Neutrophils were not observed. No granulomas were seen, and acid-fast staining and mycobacterial culture of biopsy tissue were negative.Figure 4


Enthesitis as a component of dactylitis in psoriatic juvenile idiopathic arthritis: histology of an established clinical entity.

Tuttle KS, Vargas SO, Callahan MJ, Bae DS, Nigrovic PA - Pediatr Rheumatol Online J (2015)

Characterization of cellular inflammation. Hypervascular tenosynovium showing perivascular inflammation consisting predominantly of T cells (finger; A, hematoxylin and eosin; B, CD3 immunostain; original magnification, 400×). In areas where myxoid matrix expands fibrovascular tissue containing densely collagenized tendon-like fibers, mast cells are regularly interspersed (finger; C, hematoxylin and eosin; D, CD117 immunostain; original magnification, 400×).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4359473&req=5

Fig5: Characterization of cellular inflammation. Hypervascular tenosynovium showing perivascular inflammation consisting predominantly of T cells (finger; A, hematoxylin and eosin; B, CD3 immunostain; original magnification, 400×). In areas where myxoid matrix expands fibrovascular tissue containing densely collagenized tendon-like fibers, mast cells are regularly interspersed (finger; C, hematoxylin and eosin; D, CD117 immunostain; original magnification, 400×).
Mentions: Clinical concern persisted regarding typical or atypical mycobacteria as a cause of the patient’s dactylitis, particularly in view of recent treatment with cyclophosphamide and planned tumor necrosis factor (TNF) blockade. Accordingly, deep soft tissue biopsies were performed on both swollen digits. Histologic examination of both the toe and finger showed hypervascular tenosynovium (Figure 4). Cellular inflammation (Figure 5) was mild in the toe and moderate in the finger, consisting predominantly of CD3-positive, CD5-positive, and CD4-positive T cells in both specimens, with rare CD8-positive cells. Sparse interspersed CD20-positive B cells and CD117-positive mast cells were also seen. The toe showed a small component of fibrocartilage that lacked cellular inflammation but showed reactive features including excess myxoid extracellular matrix and occasional binucleate and multinucleate chondrocytes. The finger revealed fibrinous exudate along a synovial surface and focal hypocellularity and coagulative necrosis of fibrocartilage. Neutrophils were not observed. No granulomas were seen, and acid-fast staining and mycobacterial culture of biopsy tissue were negative.Figure 4

Bottom Line: Fibrinous synovitis and mild reactive synoviocyte hyperplasia were seen.Histologic hallmarks include an expanded mast cell-populated extracellular myxoid matrix, cartilage degeneration, and a T cell-rich perivascular inflammatory infiltrate.These findings help to define enthesitis as a clinicopathologic entity.

View Article: PubMed Central - PubMed

Affiliation: Division of Immunology, Boston Children's Hospital, Boston, MA USA.

ABSTRACT

Context: Imaging of dactylitis in adult psoriatic arthritis suggests a pathophysiological role for enthesitis. However, histological definition of the dactylitic digit is unavailable.

Objective: We evaluated the role of enthesitis in dactylitis associated with psoriatic juvenile idiopathic arthritis (psJIA) in a child who underwent detailed imaging and histologic evaluation.

Design: Radiographs, ultrasound and high-resolution magnetic resonance imaging were employed to define the locus of disease in two dactylitic digits from a 14-year-old girl with psJIA. Biopsies were stained with hematoxylin and eosin, CD3, CD20, CD4, CD8 and CD117 and examined via light microscopy.

Results: Radiologic features of dactylitis included enhanced signal at digital entheses without accompanying synovitis or tenosynovitis. Histologically, finger and toe tissue exhibited hypervascular tenosynovium with a fibromyxoid expansion of fibrous tissue. This was accompanied by sparse to moderate perivascular lymphocytic inflammation consisting predominantly of T cells, with occasional admixed B cells and mast cells. Neutrophils and plasma cells were absent. Fibrocartilage exhibited reactive features including increased extracellular myxoid matrix, binucleation, and focal necrosis, without cellular inflammation. Fibrinous synovitis and mild reactive synoviocyte hyperplasia were seen.

Conclusions: Dactylitis in psJIA bears a radiographic resemblance to the condition in adult psoriatic arthritis. Histologic hallmarks include an expanded mast cell-populated extracellular myxoid matrix, cartilage degeneration, and a T cell-rich perivascular inflammatory infiltrate. These findings help to define enthesitis as a clinicopathologic entity.

Show MeSH
Related in: MedlinePlus