ABSTRACT
A 58-year-old woman who received gemcitabine for advanced gallbladder cancer developed an impaired renal function, thrombocytopenia, Raynaud's phenomenon, digital ischemic changes, a high antinuclear antibody titer and hypertensive emergency that mimicked a scleroderma renal crisis. A kidney biopsy specimen demonstrated onion-skin lesions in the arterioles and small arteries along with ischemic changes in the glomeruli, compatible with a diagnosis of hypertensive emergency (malignant hypertension). The intravenous administration of a calcium channel blocker, the oral administration of an angiotensin-converting enzyme inhibitor and angiotensin II receptor blocker and the transfusion of fresh frozen plasma were effective for treating the thrombocytopenia and progressive kidney dysfunction. Gemcitabine induces hemolytic uremic syndrome with accelerated hypertension and Raynaud's phenomenon, mimicking scleroderma renal crisis.
Subject(s)
Deoxycytidine/analogs & derivatives , Hemolytic-Uremic Syndrome/chemically induced , Hypertension, Malignant/etiology , Raynaud Disease/diagnosis , Scleroderma, Systemic/diagnosis , Antibodies, Antinuclear/blood , Blood Pressure/physiology , Deoxycytidine/adverse effects , Deoxycytidine/therapeutic use , Diagnosis, Differential , Emergencies , Female , Gallbladder Neoplasms/drug therapy , Hemolytic-Uremic Syndrome/blood , Hemolytic-Uremic Syndrome/diagnosis , Humans , Hypertension, Malignant/diagnosis , Hypertension, Malignant/physiopathology , Immunosuppressive Agents/adverse effects , Immunosuppressive Agents/therapeutic use , Kidney Glomerulus/pathology , Middle Aged , Raynaud Disease/blood , Ribonucleotide Reductases/antagonists & inhibitors , GemcitabineABSTRACT
A 55-year-old man showed a serum creatinine level of 1.51 mg/dL, CRP of 0.79 mg/dL, and proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) of 43.9 EU (normal range: below 10). The serum levels and ratios of IgG1, IgG2, IgG3, and IgG4 to total IgG were 1,570 mg/dL (49%), 1,190 mg/dL (37%), 82 mg/dL (3%), and 351 mg/dL (11%), respectively. Positron emission tomography and CT with (18)F-fluorodeoxyglucose (PET-CT) demonstrated retroperitoneal fibrosis. After a diagnosis of IgG4-related retroperitoneal fibrosis with PR3-ANCA was made, oral prednisolone improved serum creatinine and the titer of PR3-ANCA to normal levels, with no abnormal findings on PET-CT.
Subject(s)
Antibodies, Antineutrophil Cytoplasmic/blood , Immunoglobulin G/blood , Myeloblastin/immunology , Retroperitoneal Fibrosis/diagnostic imaging , Retroperitoneal Fibrosis/immunology , Creatinine/blood , Fluorodeoxyglucose F18 , Humans , Male , Middle Aged , Multimodal Imaging , Positron-Emission Tomography , Prednisolone/therapeutic use , Radiopharmaceuticals , Retroperitoneal Fibrosis/diagnosis , Retroperitoneal Fibrosis/drug therapy , Tomography, X-Ray ComputedABSTRACT
A 58-year-old Japanese woman presented with recurrent abdominal pain, chronic urticaria, and petechiae on her extremities, and hypocomplementemia, findings that were consistent with hypocomplementemic urticarial vasculitis syndrome (HUVS). A laboratory examination revealed that she had markedly elevated IgG levels (4,448 mg/dL; normal range, 870-1,700 mg/dL) with particularly high IgG4 levels (1,050 mg/dL; normal range, 48-105 mg/dL) and a high IgG4/total IgG ratio (0.22; normal range, 0.02-0.05). A skin biopsy demonstrated leukocytoclastic vasculitis with IgG4 deposition in the vascular lumen and vascular walls. A lymph node biopsy revealed reactive lymphoid hyperplasia with numerous IgG4-positive cells in the perifollicular area, but no sclerotic findings. A chromosomal analysis of an enlarged lymph node, without phytohemagglutinin (PHA) stimulation, demonstrated that one in every three analyzed cells had abnormalities, such as 44, XX, -13, add(15)(p11), -17, -17, and mar.
