Lupus nephritis associated with positive MPO-ANCA in a patient with underlying autoimmune hemolytic anemia

A 19-year-old female was admitted with general malaise and systemic edema. She had been diagnosed as having autoimmune hemolytic anemia (AIHA) eight years earlier and was successfully managed with oral prednisolone. During the current admission, she was diagnosed as having systemic lupus erythematosus (SLE) based on the presence of renal involvement, hematological abnormalities, and antinuclear and anti-double-stranded DNA antibodies, along with a recurrence of AIHA; her serology revealed a high myeloperoxydase-antineutrophil cytoplasmic antibody (MPO-ANCA) titer. She was treated with prednisolone (50 mg day⁻¹), but her renal function started to deteriorate. She responded to treatment with hemodialysis, plasmapheresis, and methylprednisolone pulse therapy; her MPO-ANCA titer and renal function improved. Treatment with intravenous cyclophosphamide gradually suppressed her AIHA and SLE activity. A renal biopsy revealed a diffuse proliferative lupus nephritis (class IV-G (A)) with necrotizing crescentic glomerulonephritis that was presumed to be associated with MPO-ANCA. The association of MPO-ANCA with SLE in this refractory case is discussed.     

Cerebral vasculitis in a patient with rheumatoid arthritis

Inflammatory vasculitis of the central nervous system is exceedingly rare in patients with rheumatoid arthritis (RA). The symptoms may be misleading. Most of the reported cases occurred in males with long-standing, nodular, destructive, rheumatoid factor-positive disease. Severe constitutional symptoms and prominent extraarticular manifestations of vasculitis were usually present. We report a case of cerebral vasculitis in a 59-year-old woman with a 20-year history of destructive rheumatoid factor-positive RA that was well controlled by methotrexate. Headache that was unresponsive to symptomatic treatment developed abruptly, together with gait disorders. Magnetic resonance imaging of the brain showed dot-like areas of high-signal in a periventricular subcortical distribution on both sides. Magnetic resonance angiography visualized a long tight stenosis of the right internal carotid artery and a string-of-beads stenosis of the left internal carotid artery suggesting vasculitis. Pulse therapy with methylprednisolone (1 g/d for 3 days) and cyclophosphamide (1 g) once a month ensured resolution of the neurological symptoms and laboratory evidence of inflammation. There was no evidence of a relapse at last follow-up after 5 months. Cerebral vasculitis is usually treated with monthly glucocorticoid and cyclophosphamide boluses separated by continuous glucocorticoid therapy. TNFalpha antagonists may be effective in patients who fail to respond to conventional treatment. However, other vasculitides such as giant cell arteritis and Wegener's granulomatosis must be ruled out, as they are refractory to TNFalpha antagonist therapy.     

The case of a patient with multiple superinfected necrosis of both legs caused by vasculitis disease

Vasculitis can lead to skin necrosis, which typically shows decayed recovery tendency and is able to lead to local as well as systemic inflammation. By repeated necrosectomy, vacuum therapy, split skin graft transplantation as well as simultaneous immunosuppression and systemic antibiotics, the cutaneous manifestations of vasculitis where cured.     

MPO-ANCA-associated pulmonary-renal vasculitis in a patient with diabetes mellitus

Diabetes mellitus is one of the major causes of chronic renal failure. Typical findings of diabetic nephropathy are early hyperfiltration followed by microalbuminuria and overt proteinuria, resulting in a progressive decrease in glomerular filtration rate. Rapidly progressive glomerulonephritis has rarely been reported in patients with diabetes mellitus. Here, we describe a patient with MPO-ANCA-associated vasculitis, presenting with pulmonary-renal syndrome. Immunosuppressive treatment, including pulse methyl-prednisolone and cyclophosphamide, was administered and the disease was resolved.     

Tubulointerstitial immune complex nephritis in a patient with cutaneous vasculitis

We report a patient who suffered from cutaneous vasculitis with renal involvement comprising tubulointerstitial (TI) nephritis and relatively mild glomerulitis. Renal biopsy revealed immune deposits both in mesangium and along tubular basement membrane (TBM), suggesting that immune complexes played a significant role in the development of renal damage. Although it is well known that vasculitic syndromes commonly affect glomeruli, this report is the first to demonstrate tubulointerstitial immune complex nephritis in a patient with cutaneous vasculitis.     

Surgical treatment in a patient with multiple systemic complications of prosthetic aortic valve endocarditis: case report

A 31-year-old woman who had undergone aortic and mitral valve replacement 1 year previously was hospitalized with suspected prosthetic valve endocarditis. Freestyle stentless aortic xenograft was successfully replaced using freestanding total aortic root replacement techniques at the left ventricular outflow tract position, and aorta-to-right coronary artery bypass was also applied with a saphenous vein graft. The patient developed multiple systemic problems during the preoperative and postoperative periods and was successfully treated with intensive interventions. She was discharged at the postoperative fourth month, and the following 28 months were uneventful.     

Perinuclear antineutrophil cytoplasmic antibody-associated vasculitis in a patient with Graves' disease treated with methimazole

A 15- year-old Caucasian girl presented with a 1-week history of necrotic ulcers on her bilateral lower extremities, associated with fever and malaise. She had had similar ulcerations on and off over the past 3 years. She had a medical history of Graves' disease, diagnosed in 1999, for which she was taking methimazole (MMI). On physical examination, there were multiple necrotic ulcers with elevated, inflamed borders and an overlying eschar on the bilateral distal extremities (Figure 1). There was one hemorrhagic, flaccid bulla on the lateral aspect of the ankle. Her complete blood count, comprehensive metabolic panel, prothrombin time, and international normalized ratio were within normal limits. Histopathology revealed a neutrophilic vasculitis involving small as well as medium-sized blood vessels. The overlying epidermis and upper dermis showed necrosis (Figure 2). Antinuclear antibody (ANA) was positive at 1:160. Perinuclear (p-) antineutrophil cytoplasmic antibody (ANCA) was positive at 1:320. Cytoplasmic ANCA was negative. The patient did not meet any clinical criteria for a diagnosis of lupus erythematosus. Direct immunofluorescence revealed IgM, complement C3, and fibrin in small- and medium-caliber blood vessels in the dermis, consistent with a leukocytoclastic vasculitis of small- and medium-caliber blood vessels (Figure 3). MMI was stopped and the patient was given prednisone, tapered over 5 days. Her thyroid function remained stable, and she did not develop new necrotic ulcers. One month later, the patient developed urticarial lesions on her lower extremities. Histopathology revealed a lymphocytic vascular reaction. No eosinophils or neutrophils were seen. These newer urticarial lesions resolved. The patient underwent debridement of the thick, necrotic ulcerative eschars. To date, her lesions have resolved (Figure 4). An ANA drawn 5 months after withdrawal of MMI was negative, and p-ANCA was 1:67.     

Pathological laughter in a patient with multiple sclerosis

Pseudobulbar affect-such as pathological laughter or crying-is associated with several different neurologic diseases and is most frequently seen in patients with Alzheimer disease. However, many physicians do not recognize it as a symptom associated with multiple sclerosis. The present report describes a case of pathological laughter in a 56-year-old man who was diagnosed as having multiple sclerosis 20 years earlier.     

Hyporeninemic hypoaldosteronism in a patient with multiple myeloma

A patient with progressive renal failure due to multiple myeloma presented with a mixed acid-base disorder (non-anion gap acidosis and respiratory alkalosis) with persistent severe hyperkalemia. Studies revealed an intact ability to lower urine pH during acid loading, markedly decreased plasma renin and aldosterone concentrations despite volume depletion, and an inappropriately low fractional excretion of potassium. Renal biopsy demonstrated plasma cell infiltration of the renal interstitium and typical proteinaceous intratubular casts. Both proximal and distal renal tubular acidification defects have been described previously in patients with multiple myeloma, but this is the first report of hyporeninemic hypoaldosteronism, hyperkalemia, and hyperchloremic metabolic acidosis in association with renal involvement in multiple myeloma.     

Polyarteritis nodosa type vasculitis in a patient with familial Mediterranean fever treated with cyclosporin A

Patients with amyloidosis secondary to familial Mediterranean fever (FMF) are known to tolerate cyclosporin A poorly. We report a case of severe cyclosporin toxicity in a patient with FMF amyloidosis who underwent kidney transplantation. The clinical syndrome consisted of severe gastrointestinal, neuromuscular, and psychiatric disturbances. Histological examination of the transplanted kidney revealed vasculitis of the polyarteritis nodosa type. We hypothesize that FMF patients are more vulnerable to the acute vascular toxicity of cyclosporin due to defective inhibition of complement activation, leading to a widespread vasculitis of the polyarteritis nodosa type.     

Spontaneous hemothorax in a patient with hereditary multiple exostoses

A case of spontaneous hemothorax in a 7-year-old child secondary to erosion of the diaphragm by an exostosis coming from the left sixth rib is reported. This rare case of hemothorax with hereditary multiple exostoses is made even rarer by the concomitant perforation of the diaphragm.