Table 2 Summary of reported cases

Morris et al. [10] - Case 1

Female

53

nd

Raynaud’s phenomenon, dysphagia, calcinosis cutis, telangiectasias

ANA

Esophageal varices, upper gastrointestinal bleeding

Barium swallow, splenoportography, liver biopsy

End-to-side porto-caval anastomosis

Abscence of complications in 12 months

Morris et al. [10] - Case 2

Female

49

nd

Raynaud’s phenomenon, sclerodactily

ANA

Anemia, ascites

esophageal varices, upper gastrointestinal bleeding

Barium swallow, splenoportography, liver biopsy

End-to-side porto-caval anastomosis, diuretics

Recovered from ascites

Umeyama et al. [11]

Female

41

Limited Cutaneous Scleroderma

Microstomy, sclerodactily, esophagopathy

ANA

Pancytopenia, hepatosplenomegaly, upper gastrointestinal bleeding,

collateral circulation,

esophagogastric varices

Upper gastrointestinal endoscopy, splenoportography

Splenectomy,

esophageal transection

nd

Kabukari et al. [12]

Female

33

nd

Raynaud’s phenomena, sclerodactily, digital pitting scars, microstomy, interstitial lung disease, chronic kidney disease

ANA, anti-U1-RNP, Anti-SSA-Ro, Anti-SSB-la

Thrombocytopenia, hepatoesplenomegaly, ascites, collateral circulation

Percutaneous transhepatic portography with venous pressure measurement,

liver biopsy

Prednisolone 30 to 60 mg, intravenous immunoglobulin, diuretics

Death by renal and hepatic disfunction

Manuel et al. [13]

Female

54

Limited Cutaneous Scleroderma

Calcinosis cutis, raynaud’s phenomenon, esophagopathy, sclerodactily, telangiectasias

ANA, anticentromere

Anemia, thrombocytopenia, esophageal varices, upper gastrointestinal bleeding, splenomegaly, portal hypertension gastropathy

Abdominal doppler ultrasound, upper gastrointestinal endoscopy, arteriography, liver biopsy

Endoscopic sclerosis of esophageal varices, propranolol,

portosystemic shunt

Without gastrointestinal bleeding in 1 year

Garcia et al. [14]

Male

59

Diffuse Cutaneous Scleroderma

nd

None

Hepatomegaly, splenomegaly, telangiectasias, palmar erythema, esophageal varices, portal hypertension gastropathy, thrombocytopenia

Abdominal ultrasound, computed tomography, resonance, upper gastrointestinal endoscopy, liver biopsy, portography

nd

nd

Tsuneyama et al. [15] - Case 1

Female

61

Diffuse Cutaneous Scleroderma

nd

ANA

Anemia, ascites, splenomegaly

esophageal varices, upper gastrointestinal bleeding

Liver biopsy

nd

Death by pneumonia

Tsuneyama et al. [15] - Case 2

Female

58

Diffuse Cutaneous Scleroderma

Interstitial lung disease, telangiectasias

ANA, anti-SCL70

Anemia, esophageal varices, splenomegaly, ascites

Liver biopsy

Splenectomy,

esophageal dissection

Death by sepsis 4 months later

Kogawa et al. [16]

Female

72

Limited Cutaneous Scleroderma

Raynaud’s phenomenon, interstitial lung disease, telangiectasias

ANA, anti-SCL70, anticentromere, anti-SSA-Ro

Esophageal varices, anemia, thrombocytopenia

Computed tomography, liver biopsy, upper gastrointestinal endoscopy

Oral corticosteroids

Death by

respiratory failure

due to de ILD

exacerbation

Moschos et al. [17]

Male

82

nd

Interstitial lung disease

nd

Mild anemia, esophageal varices, upper gastrointestinal bleeding

Upper gastrointestinal endoscopy, ultrassound,

computed tomography,

Liver biopsy

Propranolol,

azathioprine,

spironolactone,

prednisolone,

lansoprazole, endoscopic varices sclerotherapy and banding, Sengstaken blakemore tube, intravenous glypressin

Stable in 2 months

Takagi et al. [18]

Female

62

nd

Raynaud’s phenomenon, sclerodactily, digital pitting scars, interstitial lung disease

ANA, anti-U1-RNP

Pancytopenia, ascites, pleural effusion, splenomegaly

esophageal varices

Ultrassound,

computed tomography, magnetic resonance imaging,

liver biopsy

Prednisolone 30 mg, diuretics

Stable 3 years later

Samanta et al. [1]

Male

65

Limited Cutaneous Scleroderma

Raynaud’s phenomenon, calcinosis cutis, sclerodactily, esophageal disease, telangiectasias

ANA, anticentromere, Rheumatoid factor

Esophageal varices, upper gastrointestinal bleeding, portal gastropathy

Ultrassound,

contrasted computed, tomography,

liver biopsy, upper gastrointestinal endoscopy

Hemodynamic resuscitation,

endoscopic esophageal varices band ligation, intravenous glypressin

nd

Espinosa et al. [19]

Female

38

Limited Cutaneous Scleroderma

Raynaud’s phenomenon, sclerodactily, interstitial lung disease, telangiectasias, arthritis, esophagopathy, pulmonary hypertension, peripheral neuropathy

ANA, anticentromere

Esophageal varices, splenomegaly

Ultrassound, liver biopsy, magnetic resonance imaging

Endoscopic ligation of esophageal varices, prophylactic treatment with 40 mg of propranolol every 12 h.

nd

Kamel et al. [20]

Female

57

Sin scleroderma

Pulmonary arterial, hypertension

ANA, anti-ds-DNA, anticentromere, Anti-polimerase III

Gastroesophageal varices

Computed tomography, liver biopsy

Diuretics, ambrisentan, sildenafil, furosemide, oxygen supplementation, hydroxychloroquine

Cardiac sudden death one year later

Abrams et al. [21]

Male

59

Limited Cutaneous Scleroderma

CREST syndrome (calcinosis, Raynaud’s phenomenon, esophagopathy, sclerodactily, telangiectasias)

ANA

Ascites,

esophageal varices, pleural effusions

Abdominal ultrassound,

liver biopsy

Diuretics, thoracocentesis, paracentesis, transjugular intrahepatic portal systemic shunt study (TIPS)

Improvement of

ascitis and

pleural effusion

after TIPS.

Encephalophaty

controlled

with medication

Gao et al. [3]

Female

51

nd

Raynaud’s phenomenon, sclerodactily, puffy fingers

ANA, anticentromere

Anemia, esophageal varices, upper gastrointestinal bleeding

Doppler ultrasound, computed tomography

Splenectomy

nd

Saigusa et al. [22]

Female

73

Limited Cutaneous Scleroderma

Raynaud’s phenomenon, sclerodactily, digital ulcers, lower limbs ulcers, pulmonary arterial hypertension

ANA, anticentromere, anti-SSA-Ro

Anemia, esophageal varices

Upper gastrointestinal endoscopy, computed tomography

Endoscopic esophageal varices band ligation

Death by

deterioration of

her condition

Yamamoto et al. [23]

Female

76

Limited Cutaneous Scleroderma

Raynaud’s phenomenon, sclerodactily

ANA, anticentromere, Anti-ds-DNA

Mild anemia, thrombocytopenia, ascites,

esophageal varices

Computed tomography, upper gastrointestinal endoscopy,

magnetic resonance imaging, liver biopsy

Diuretics

No symptoms of ascitis or SSc

Colaci et al. [2]

Female

61

Limited Cutaneous Scleroderma

Telangiectasias, esophagopathy

ANA, anticentromere

Ascites

collateral circulation

Liver biopsy, computed tomography, upper gastrointestinal endoscopy

Diuretics

Stable for 1 year

follow-up.

Death by sepsis

later

Hitawala et al. [24]

Female

37

Limited Cutaneous Scleroderma

Raynaud’s phenomenon, sclerodactily, telangiectasias, interstitial lung disease

ANA, anticentromere, Rheumatoid factor

Splenomegaly, pancytopenia,

gastroesophageal varices, upper gastrointestinal bleeding,

collateral circulation,

ascites

Ultrassound,

computed tomography, upper gastrointestinal endoscopy, hepatic elastography, magnetic resonance imaging, liver biopsy

Endoscopic esophageal varices band ligation, carvedilol

Stable 1 year later

Present Case

Female

50

Diffuse Cutaneous Scleroderma

Raynaud’s phenomenon, puffy fingers, myositis, interstitial lung disease, esophageal reflux, scleroderma renal crisis

ANA

Mild anemia, ascites,

esophageal varices,

collateral circulation

Ultrassound, computed tomography,

magnetic resonance imaging, upper gastrointestinal endoscopy, hepatic elastography, liver biopsy

Antihypertensives

diuretics,

endoscopic esophageal varices band ligation

Stable 5 years later