X-Ray Diffraction of Spontaneously Draining Calcinosis in Patients with Scleroderma

Vivien Hsu¹, Thomas Emge² and Naomi Schlesinger³, ¹Medicine, Rutgers University, New Brunswick, NJ, ²Dept of Chemistry and Chemical Biology, Rutgers- the State University, Piscataway, NJ, ³Medicine, Rutgers-Robert Wood Johnson Medical School, New Brunswick, NJ

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Calcinosis and scleroderma

Session Information

Date: Tuesday, November 10, 2015

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's - Clinical Aspects and Therapeutics Poster III

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Calcinosis is caused by deposition of calcified materials in the soft tissues (1). Hydroxyapatite (HA) is reported to be its major constituent (2). Mechanical stress and local tissue hypoxia are believed to be important in its pathogenesis (3). Our aim was to analyze spontaneously draining material from calcinosis sites in scleroderma (SSc) patients using x-ray diffraction.

Methods: In this IRB-approved study, we enrolled SSc patients meeting the American College of Rheumatology criteria for definite SSc (4). Pertinent clinical data was collected. Xray diffraction data were used to determine solid phase present (e.g., HA versus other Ca phosphate phase) and to approximate the % amorphous and % crystalline components using a Bruker HiStar multi-wire area detector.

Results: Ten female subjects (see Table 1) with advanced SSc were enrolled with mean disease duration 16 years; 6 had diffuse SSc. Calcinosis occurred later in the disease course and 7 had extensive calcinosis affecting multiple sites. Draining calcinosis was collected from multiple sites, most commonly the hand. X-ray diffraction confirmed HA of varying percentages in all but one specimen. Solid samples generally contained higher amounts of HA.

Conclusion: By using x-ray diffraction, our study corroborates previous published reports (2, 3) that HA crystal deposition is the main constitute of SSc-related calcinosis. Solid samples contained higher amounts of HA crystals; fluid samples observed by optical microscopy to contain solids, including HA in suspension. Further research is needed to characterize the amorphous materials associated with HA deposition in SSc patients with calcinosis.

Table 1: SSc clinical characteristics and crystal analysis:

Patient #	SSc type	SSc/ calcinosis onset (in years)	Auto-antibodies	sites	State	Crystalline component
1	D*	15/9	ANA/Nucleolar/ Scl70	elbow thigh groin	liquid* liquid liquid	HA** (25%) HA (20%) HA (20%)
2	D	19/9	ANA/Scl70	belly	liquid	HA (3%)
3	L**	15/3	ANA/ACA	groin	liquid	HA (2%)
4	L	19/5	ANA/ACA/RNP	finger	liquid	HA (8%)
5	L	16/3	ANA	Elbow over 3 days	solid liquid solid	HA (40%) HA (40%) HA (4%)
6	D	14/3	Scl70/RNApol3	finger	solid	HA (15%)
7	D	11/9	Scl70	finger over 3 days	solid liquid liquid	HA (50%) HA (7%) HA (10%)
8	D	9/4	Scl70/ANA	shoulder	Solid mass	HA (3%)
9	L	29/4	ANA/RNApol3/ nucleolar	Belly finger	Solid*** solid	calcite (<1%) HA (2%)
10	D	10/2	ANA	finger	solid	HA (43%)

*Diffuse **Limited

References

Steen et al Arthritis Rheum 1984; 27:125.
Leroux et al. J Rheumatol 1983; 10:242.
Davies et al. Rheumatology 2009; 48:876.
Hoogen et al., Ann Rheum Dis 2013;72:1747 & Arthritis Rheum 2013;65(11):2737-57.

Disclosure: V. Hsu, None; T. Emge, None; N. Schlesinger, Novartis Pharmaceutical Corporation, 2,Takeda, novartis, 8,Novartis, Astra Zeneca, Pfizer, 5.

To cite this abstract in AMA style:

Hsu V, Emge T, Schlesinger N. X-Ray Diffraction of Spontaneously Draining Calcinosis in Patients with Scleroderma [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/x-ray-diffraction-of-spontaneously-draining-calcinosis-in-patients-with-scleroderma/. Accessed .

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