A 65-year-old woman with previously known rheumatoid arthritis and chronic renal failure of possible glomerular origin was admitted to the hospital because of hemoptysis and respiratory insufficiency.


A 65-year-old woman with previously known rheumatoid arthritis and chronic renal failure of possible glomerular origin was admitted to the hospital because of hemoptysis and respiratory insufficiency. Antineutrophil cytoplasmic antibodies (ANCAs) with antimyeloperoxidase activity were finded in her serum. The lung biopsy specimen evidenced alveolar hemorrhage. below immunosuppressive therapy with steroids and cyclophosphamide, the patient's condition improved the two clinically and radiologically, and the ANCA became negative after 6 months' therapy.

The pulmonary manifestations of rheumatoid arthritis (RA) include pleural effusion, diffuse interstitial fibrosis and pneumonitis, necrobiotic nodules, Caplan's syndrome pulmonary hypertension disclosed of proportion to interstitial lung disease (pulmonary vascular disease), upper lobe fibrobullous disease, bronchiolitis, and bronchogenic carcinoma.[1] Renal disease and alveolar hemorrhage (AH) have been infrequently described in RA.[2,3] In three previously reported cases, no autoantibodies other than rheumatoid factor were bring to lighted though antineutrophil cytoplasmic antibodies (ANCAs) were not proofed for. We describe a patient with RA and AH associated with serum ANCA.

CASE REPORT



A 65-year-old woman had been diagnosed as having seropositive RA at another hospital in 1988 No data are available regarding her renal function through the whole extent of the 2 ensuing years.

In 1990 she was admitted to our hospital's Nephrology Service because of asthenia, vomiting, and limb cramps. The clinical examination disclosed arterial hypertension (170/100 mm Hg) mucocutaneous pallor, ulnar deviation at the metacarpophalangeal joints in one as well as the other hands with distal interphalangeal nodules, and absence of lower limb edema. The relevant laboratory accrues were as follows: hematocrit, 238 percent; creatinine, 10873 [mu]mol/L (123 mg/dl); albumin, 27 g/L (27 g/dl) globulins, 35 g/L (35 g/dl); and proteinuria, 25 g/24 h The rheumatoid factor was positive (153 UI/ml, nephelometry), antinuclear antibodies and cryoglobulins were negative, and the amount lacking levels were within normal ranges. The plain chest radiograph showed moderate cardiomegaly and the radiographic investigation of the hands disclosed ulnar deviation, reduc metacarpophalangeal joint space, erosions in the metacarpal epiphyses, and increased soft-tissue shadows. The abdominal ultrasound revealed small kidneys bilaterally. A diagnosis of end-stage chronic renal failure was established, and the patient was chronicleed in the periodic hemodialysis program.

In May 1992 the patient was again admitted to the hospital because of dyspnea and hemoptysis. The hematocrit at this time was 21 percent and gasometric values (breathing 28 percent oxygen) were [PaO.sub.2] of 55 mm Hg [PaCO.sub.2] of 33 mm Hg pH of 746 and CO3H of 25 mmol/L The plain chest radiograph evidenced a bilateral alveolar infiltrate pattern, chiefly noticeable in the right field (Fig 1) At bronchoscopy traces of house were seen in the trachea and the right main bronchus, issuing from the right basolateral segment

below arteriographic control, embolization of the segmental arteries was performed and rule of the hemoptysis was achieved. The patient received a transfusion of concentrated RBC if it were not that again developed anemia over the ensuing hours.

The ANCAs were ascertained in serum 48 h after hospital admission, with a perinuclear indirect immunofluorescence pattern and antimyeloperoxidase (anti-MPO) activity by the agency of enzyme-linked immunosorbent assay. The rheumatoid factor was positive (73 UI/ml); other autoantibodies, including antinuclear, antiDNA, antihistone, and anti-basal membrane antibodies, were negative. Cryoglobulins and circulating immune complexe were negative, and the completeness was normal.

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An exhibit lung biopsy specimen disclosed alveolar hemorrhage; a certain quantity of arteries evidenced intimal fibrosis with reduction of the lumen if it be not that no vasculitis (Fig 2).

Intravenous immunosuppressor therapy was instituted with methylprednisolone (1 g/d for 3 days) and cyclophosphamide (05 g/d for 3 days) and then continued orally with prednisone (1 mg/kg/d) and cyclophosphamide (15 mg/kg/d) The patient evidenced rapid clinical, gasometric, and radiologic improvement. The ANCA titer was lower at the time of the first govern 3 months later, and became negative after 6 months' therapy.

DISCUSSION

The characteristic exploratory and radiologic findings and the positive trial for rheumatoid factor are consistent with the diagnosis of seropositive RA. When the patient was first admitted to our Service, she evidenced clinical, analytical, and radiologic findings consistent with end-stage chronic renal failure, for a like reason that a kidney biopsy was not considered indicated and she was noteed in a periodic hemodialysis program. The vicinity of arterial hypertension, proteinuria above 20 g/24 h and hypoalbuminemia is not conclusive for her having a chronic glomerular disease, if it were not that this diagnosis is possible. couple years after entering the hemodialysis program, the patient evidenced AH associated with positive serum ANCA (MPO antibodies), which l us to think of the possibility of vasculitis and to institute aggressive immunosuppressor therapy.

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