Scleroderma renal crisis and concurrent isolated pulmonary hypertension in mixed connective tissue disease and overlap syndrome: report of two cases.
We here report on scleroderma renal crisis (SRC) appearing concurrently with isolated pulmonary hypertension (IPHT), that is, pulmonary hypertension without interstitial lung disease with fibrosis, in a patient with mixed connective tissue disease (MCTD) and in one with overlap syndrome or undifferentiated connective tissue disease (UCTD). To the best of our knowledge there are only five previous reports on SRC in MCTD and UCTD. The unexpected appearance of SRC in the setting of concomitant IPHT and limited or absent scleroderma skin changes is discussed.
Andersen
G N
GN
Department of Rheumatology, University Hospital of Umeå, Sweden. grethe.andersen@obbit.net
Vasko
J
J
D058186:Acute Kidney Injury / Q000150:complications* / Q000175:diagnosis; D000328:Adult; D001707:Biopsy, Needle; D003240:Connective Tissue Diseases / Q000150:complications / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000150:complications* / Q000473:pathology; D018570:Risk Assessment; D012595:Scleroderma, Systemic / Q000150:complications* / Q000473:pathology; D012720:Severity of Illness Index