ABSTRACT
A 70-year-old man presented with 1 month of haematuria and mild right-sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of antimyeloperoxidase antibodies, a kidney biopsy which demonstrated necrotising crescentic glomerulonephritis with linear immunofluorescence of the basement membrane, and electron microscopy which exhibited thickening of the glomerular basement membrane. Incidentally, the patient was discovered to have a latent hepatitis B infection, which complicated immunosuppressive therapy. He was treated with a course of plasmapheresis and methylprednisolone, followed by entecavir for hepatitis B prophylaxis, and finally by rituximab. This case of glomerulonephritis was notable for its resemblance to the better known Goodpasture's disease. Typically, Goodpasture's syndrome exists on a spectrum from seronegative disease to double-positive disease that presents with both anti-glomerular basement membrane (anti-GBM) and cytoplasmic-antineutrophil cytoplasmic antibodies/antiproteinase 3 antibodies (c-ANCA/anti-PR3). However, this patient's glomerulonephritis was unique because he presented negative for anti-GBM antibodies and positive for perinuclear-antineutrophil cytoplasmic antibodies/antimyeloperoxidase antibodies (p-ANCA/anti-MPO).
Subject(s)
Glomerulonephritis/diagnosis , Lung Diseases, Interstitial/diagnosis , Peroxidase/immunology , Aged , Antibodies, Antineutrophil Cytoplasmic/metabolism , Autoantibodies/metabolism , Diagnosis, Differential , Glomerulonephritis/immunology , Glomerulonephritis/therapy , Guanine/analogs & derivatives , Guanine/therapeutic use , Humans , Lung Diseases, Interstitial/immunology , Lung Diseases, Interstitial/therapy , Male , Methylprednisolone/therapeutic use , Microscopy, Electron , Plasmapheresis , Rituximab/therapeutic use , Treatment OutcomeABSTRACT
We analyzed quantitative maps of T1 and T2 relaxation times and muscle fat fraction measurements in magnetic resonance imaging of the upper arm skeletal muscles and heart in ambulatory boys with Duchenne muscular dystrophy and age-range-matched healthy volunteer boys. The cardiac-optimized sequences detected fatty infiltration and edema in the upper arm skeletal muscles but not the myocardium in these Duchenne muscular dystrophy boys who had normal ejection fraction. Imaging the heart and skeletal muscle using the same magnetic resonance imaging methods during a single scan may be useful in assessing relative disease status and therapeutic response in clinical trials of Duchenne muscular dystrophy.