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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

Magnetic resonance imaging evaluation of dactylitis. A. Coronal short tau inversion recovery (STIR), B. sagittal T2 with fat suppression, and C-F. axial T2 with fat suppression of the left fifth toe, proximal to distal sequences, demonstrating increased signal and gadolinium enhancement at the local entheses without associated tendonitis or synovitis.
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Fig3: Magnetic resonance imaging evaluation of dactylitis. A. Coronal short tau inversion recovery (STIR), B. sagittal T2 with fat suppression, and C-F. axial T2 with fat suppression of the left fifth toe, proximal to distal sequences, demonstrating increased signal and gadolinium enhancement at the local entheses without associated tendonitis or synovitis.

Mentions: Due to the concern over possible infectious dactylitis, including tuberculous [19], the patient underwent an unusually comprehensive evaluation. Chest radiograph, purified protein derivative test, sputum acid fast stain, and sputum mycobacterium culture were negative. Radiographs of the right hand and left foot showed soft tissue swelling of the right fourth digit and left fifth toe, without erosions or periosteal reaction (Figure 2A-C). Magnetic resonance imaging studies of the left fifth toe revealed swelling and contrast enhancement at the flexor tendon insertion with associated bone marrow edema, consistent with enthesitis, in the absence of either synovitis or tenosynovitis (Figure 3).Figure 2


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)

Magnetic resonance imaging evaluation of dactylitis. A. Coronal short tau inversion recovery (STIR), B. sagittal T2 with fat suppression, and C-F. axial T2 with fat suppression of the left fifth toe, proximal to distal sequences, demonstrating increased signal and gadolinium enhancement at the local entheses without associated tendonitis or synovitis.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig3: Magnetic resonance imaging evaluation of dactylitis. A. Coronal short tau inversion recovery (STIR), B. sagittal T2 with fat suppression, and C-F. axial T2 with fat suppression of the left fifth toe, proximal to distal sequences, demonstrating increased signal and gadolinium enhancement at the local entheses without associated tendonitis or synovitis.
Mentions: Due to the concern over possible infectious dactylitis, including tuberculous [19], the patient underwent an unusually comprehensive evaluation. Chest radiograph, purified protein derivative test, sputum acid fast stain, and sputum mycobacterium culture were negative. Radiographs of the right hand and left foot showed soft tissue swelling of the right fourth digit and left fifth toe, without erosions or periosteal reaction (Figure 2A-C). Magnetic resonance imaging studies of the left fifth toe revealed swelling and contrast enhancement at the flexor tendon insertion with associated bone marrow edema, consistent with enthesitis, in the absence of either synovitis or tenosynovitis (Figure 3).Figure 2

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