Retroperitoneal Fibrosis and Deep Vein Thrombosis in a Patient With Scleroderma

A 68-year-old man presented with edema in his left leg and thickened skin of both legs persisting for 5 years. Computed tomography (CT) showed thickened soft tissues of the pelvic wall (Fig. 1). The diagnosis of retroperitoneal fibrosis with scleroderma was established. Physical examination revealed thickened and hardened skin of both lower limbs. C-reactive protein (CRP) level, erythrocyte sedimentation rate (ESR), and serum immunoglobulin G4 level were within the normal limits (2.88 mg/L, 22 mm/h, and 49 mg/dL, respectively).

FIGURE 1.

Thickened soft tissues: retroperitoneal (black arrow), anterior to the sacrum (arrowhead), and left and right pelvic wall (white arrow).

Antinuclear antibodies, antiphospholipid antibodies, anti-Scl-70 antibodies, and human leukocyte antigen B27 were negative. Skin biopsy confirmed the presence of scleroderma (Fig. 2). After the diagnosis, we prescribed methylprednisolone (40 mg, once per day) and cyclophosphamide (0.4 g, every 2 weeks). The dosages of both drugs were gradually reduced. At 6 months after the treatment, the lower-limb edema had improved and the skin had softened. Abdominal CT showed significantly reduced presacral and retroperitoneal lesions (Fig. 3a). The patient was treated with corticosteroids for 1 year and intermittent cyclophosphamide for 5 years after discharge. Follow-up evaluations were performed at 3, 6, and 9 years (Fig. 3b–d).

FIGURE 2.

Excessive collagen fiber hyperplasia and swelling in the skin derma (arrowhead), atrophied skin appendage surrounded by collagen fibers (black arrow), and thickened walls of small vessels with lymphocytic infiltration (white arrow). Hematoxylin-eosin staining, original magnification ×100.

FIGURE 3.

A, Six months after treatment: no obvious thickening in the intestinal wall (black arrow) and no soft tissue mass in the pelvic cavity; thickened soft tissue of the presacral region (arrowhead) and left and right pelvic wall (white arrow), which was more obvious on the left side. B, Three years after treatment: thickening of soft tissue anterior to the sacrum (arrowhead) was alleviated. C, Six years after treatment: similar to (B). D, Nine years after treatment: thickened soft tissues of the anterior (white arrow) and left and right pelvic wall.

At 6 years (1 year after cyclophosphamide withdrawal), the patient developed deep vein thrombosis (DVT) in his left lower limb and was treated with anticoagulants (Fig. 4). At 9 years, abdominal CT showed the development of mild retroperitoneal soft tissue fibrosis (Fig. 3d).

FIGURE 4.

A, Total occlusion of the left external iliac vein (white arrow). B, Partial occlusion of the left femoral vein (white arrow).

This is the first case in which DVT developed after retroperitoneal fibrosis and scleroderma. Previous studies have proposed some prognostic predictors of scleroderma, including ESR and CRP level.¹ However, ESR and CRP level failed to predict the prognosis in this case. Patients with scleroderma may have a high risk of DVT, especially in the first year after the diagnosis.² However, DVT may not occur immediately after the appearance of scleroderma. In this case, corticosteroids combined with cyclophosphamide effectively and rapidly controlled the disease progression. Prolonged immunosuppressive therapy may be more beneficial. CT is a useful method for locating lesions and evaluating disease progression or remission.^3,4