Systematic review of fibroblastic rheumatism: A case report

Table 1 Patient characteristics included in the systematic review

Variables	Types	No. (n = 33)	%
Gender	Man	22	66.67
	Female	11	33.33
Age	0-19	12	36.36
	20-39	3	9.09
	40-59	13	39.39
	≥ 60	5	15.16
Presenting symptoms	Cutaneous periarticular nodules on the hands	28	84.85
	Cutaneous periarticular nodules on the feet	13	39.39
	Cutaneous periarticular nodules on others parts	9	27.27
	Sclerodactyly	19	57.58
	Raynaud’s phenomenon	9	27.27
	Redness and swelling	13	39.39
	Polyarthritis (classically affecting distal joints)	19	57.58
	Arthralgias	19	57.58
	Tendonitis	10	30.3
	Thickened palmar/plantar fascia	10	30.3
	Joint contractures	12	36.36
Diagnostic imaging tests	Acroosteolysis	16	48.48
	Erosive arthropathy	13	39.39
	Osteoproliferation	3	9.09
	Joint space stenosis	12	36.36
Histologic features	Absence or decrease of elastic fibers	9	27.27
	Increased proliferation of dermal fibroblasts	24	72.72

Table 2 Immunohistochemical findings in patients with fibroblastic rheumatism with available data

Immunohistochemical markers	Reactivity	Number of patients with available data (n/N, %)
CD34	+	5/20 (62.5)
	-	15/20 (62.5)
S100	+	4/23 (71.88)
	-	19/23 (71.88)
EMA	+	1/10 (31.25)
	-	9/10 (31.25)
α-SMA	+	25/28 (87.5)
	-	3/28 (87.5)
Vimentin	+	18/18 (56.25)
	-	0/18 (56.25)
CD68	+	2/12 (37.5)
	-	10/12 (37.5)

α-SMA: α-smooth muscle actin; EMA: Epithelial membrane antigen.

Table 3 Laboratory findings in patients with fibroblastic rheumatism with available data

Laboratory data	Expressed levels	Number of patients with available data (n/N, %)
ANAs	+	3/31 (96.88)
	-	28/31 (96.88)
ds-DNA	+	0/18 (56.25)
	-	18/18 (56.25)
ANCA	+	3/23 (71.88)
	-	20/23 (71.88)
Anti-SSB	+	1/15 (46.88)
	-	14/15 (46.88)
Anti-Scl-70	+	0/17 (53.13)
	-	17/17 (53.13)
Anti-Jo-1	+	0/21 (65.63)
	-	21/21 (65.63)
C3	+	0/13 (40.63)
	-	13/13 (40.63)
RF	+	0/33 (100)
	-	33/33 (100)
CRP	+	4/25 (78.13)
	-	21/25 (78.13)

ANAs: Antinuclear antibodies; ANCA: Anti-neutrophil cytoplasmic antibodies; RF: Rheumatoid factor; CRP: C reactive protein.

Table 4 Key points of differential diagnosis between various similar diseases

	RA	PsA	MCRH	NS	FR
Clinical features
Skin
Sclerodactyly	+/-	-	-	+	+
Subcutaneous nodules	+	-	+	-	+
Raynaud phenomenon	+	-	-	-	+
Musculoskeletal
Favorite sites
DIP	-	+	+	+	+
PIP	+	+	+	+	+
MCP	+	+/-	+	+/-	+
Histopathologic features
Proliferation of fibroblasts	-	-	-	+	+
Proliferation of collagen fibers	-	-	-	+	+
Multinucleated giant cells	-	+	+	+	+/-
Synovitis	+	+	+	+/-	+
Loss of elastin	-	-	+	-	+
Immunohistochemistry
CD34	+/-	+	+/-	+	+/-
SMA	-	+/-	+/-	+/-	+
Vimentin	-	-	-	-	+
C3	+	+	+/-	+/-	-
Other features
Elevation of CRP and/or ESR	+	+	+/-	+	-
Rheumatoid factor	+	-	-	+/-	-
Redness and swelling	+	+/-	-	+/-	+

RA: Rheumatoid arthritis; PsA: Psoriatic arthritis; NS: Nodular scleroderma; MCRH: Multicentric reticulohistiocytosis; FR: Fibroblastic rheumatism; DIP: Distal interphalangeal joint; PIP: Proximal interphalangeal joint; MCP: Metacarpophalangeal joints; SMA: Smooth muscle actin; CRP: C-reactive protein; ESR: Erythrocyte sediment rate.