Diffuse scleroderma diagnosed 30 years ago
History of scleroderma renal crisis 24 years ago
Severe Raynaud’s and recurrent digital ischemic ulcers s/p amputations
Diffuse skin disease that has improved since time of diagnosis
Severe GI disease with chronic diarrhea and weight loss. Has required multiple courses of antibiotics and fecal transplants and disease complicated by multiple episodes of c.diff.
Recurrent pericardial effusions
Interstitial lung disease stable on most recent chest CT
Musculoskeletal disease with flexion contractures in both hands and elbows. +inflammatory arthritis and history of tendon friction rubs

 

surgery

PERICARDIUM

Final Diagnosis

PERICARDIUM:
FIBROCONNECTIVE TISSUE WITH FIBROSIS AND ASSOCIATED REACTIVE MESOTHELIUM WITH MILD CHRONIC INFLAMMATION AND FIBRINOUS EXUDATE.
NO TUMOR IDENTIFIED.

Still have to upload all pictures from pic library on work (home computer in folder called acroosteolysis

CXR

Heart and mediastinum: The cardiomediastinal silhouette is stable
without significant cardiomegaly.

Lungs and pleura: Lungs are hyperinflated. Scarring at the bilateral
lung apices. Lungs are otherwise clear. No pneumothorax. No pleural
effusions

 

 

4 years ago

Presented with a large pericardial effusion, significantly increase in volume since J1 month prior, of uncertain
most likely chronic given the placement of a
pericardial drainage in the past therefore likely associated with
connective tissue disease. Prior pericardial drain has been removed.

Echocardiogram

Showed a large  circumferential pericardial  effusion  with
significant fibrinous  exudate adherent  to the epicardial  surface.
Delayed diastolic  expansion of the  distal RV suggested  raised
intrapericardial  pressure.

IVC size was normal with  blunted respirophasic  variation  suggestive of high normal RA pressure  (5-10 mm Hg).

Pericardial window followed

Path report

Pericardium:
Fibroconnective tissue with fibrosis and associated reactive mesothelium with mild chronic inflammation and fibrinous exudate.
No tumor identified.

 

CT of the Chest

• Mild groundglass opacities predominating within the peripheral /subpleural lung apices and lower lobes bilaterally.
• multiple bilateralpulmonary nodules.Pleura: No pleural effusion.Heart and pericardium: No pericardial effusion. There is a
  pericardial drain with its tip terminating adjacent to the pulmonary
  trunk. There is trace air within the peritoneum where the pericardial
  drain is exiting from the body inferior to the sternum.

 

Dilated Esophagus

Soft Tissue Calcification and Ossification

 

Acro-osteolysis, Contraxctures and Soft Tissue Calcification

Right HAnd

• soft tissue swelling about the 3rd digit.
• dDystrophic calcifications  about the
  distal phalanges along the medial aspect of the 5th metacarpal.
•  joint space narrowing involving the 2nd through 5th DIPs. contracture deformities
  are compatible with a history of scleroderma.

 

 

 

 

 

Left Hand

Severe acroosteolysis of the third digit to the level of the distal
metaphysis of the proximal phalanx. There is also acroosteolysis of
the thumb and index fingers. Scattered regions of soft tissue
calcification in the tuft of the thumb and in the proximal soft
tissues of the index finger. Narrowing of the remaining IP joints
with periarticular osteopenia. No acute fracture. Alignment is
anatomic.
Findings suggestive of scleroderma

Ballcatchers view

severe acroosteolysis of the middle finger to the
level of the proximal phalanx distal metaphysis, consistent with
known history of scleroderma. There is also acroosteolysis of the
thumb distal phalanx and of the index finger to the level of the
middle phalanx. Scattered areas of soft tissue calcification in the
tuft of the thumb and in the proximal soft tissues of the index
finger. Mild to moderate narrowing of the remaining IP joints. No
acute fracture. No marginal erosions.

• TCV
  • Scleroderma Introduction
    • Scleroderma Cases
      • 001Lu  Scleroderma NSIP and Calcinosis of the Spine
      • 050Lu 28f Scleroderma NSIP Acro osteolysis 
      • 110Lu NSIP and Scleroderma
      • 118Lu Scleroderma and Aspiration Pneumonia
      • 126Lu Scleroderma ILD Acroosteolysis Pericardial Effusion
      • 142Lu scleroderma no lung disease ST calcification