A 40-year-old woman, previously healthy, Colombian, was admitted to the Department of Rheumatology at Hospital Militar Central, with a fever of 39 C, generalised arthralgia involving the elbows, wrists, proximal and distal interphalangeal joints, metacarpophalangeal and metatarsophalangeal joints, knees and ankles, and fatigue. She had been in her usual state of good health until 2 weeks before admission and these symptoms starting shortly after the patient had a sore throat for 7 days.
Examination revealed angioneurotic edema detected on soles, palms and tongue (Figure 1a) and widespread red, urticated plaques in a symmetrical distribution affecting the arms, dorsal hands, upper and lower chest and back (Figure 1b). The rest of the examination was unremarkable. These lesions were present for less than 10 h per day and were not more pronounced at night with no associated bruising. She was diagnosed as having infection-associated-urticaria in a peripheral hospital and was treated with antibiotics (ciprofloxacin) and antihistamines (loratadine) without diminishment of her symptoms. Followed 5 days later by fever, the patient presented dyspnea, cough and hypoxemia. A chest x-ray film showed a patchy right lower lobe infiltrate (Figure 2a) and computed tomography showed unilateral consolidation and pleural effusion (Figure 2b).
An early bronchoscopy with bronchoalveolar lavage was indicated revealing hemorrhagic lavage and pathological examination showed hemosiderin-containing macrophages in 80%. Bronchoalveolar lavage specimen was sent for routine bacterial cultures, fungal, viral and Pneumocystis carinii; all of the results were negative. A skin biopsy demonstrated dermal oedema, and a marked perivascular and neutrophilic infiltrate with no evidence of structural vasculitis. These features were consistent with neutrophilic urticaria (Figure 3). Other investigations revealed a normocytic anaemia Hb 11.5 g/dL erythrocyte mean corpuscolar volume 86 fl platelet count 408.000/mm3, a marked white blood cell count (WBC) 67.200 mm3 with 91% neutrophils, 5% lymphocytes, 1% eosinophils, 3% monocytes and markedly elevated acute phase reactants: erythrocyte sedimentation rate 49 mm in the first hour, C-reactive protein 11,43 mg/dl and ferritin 1650 mug/L, alanine aminotransferase was mildly elevated at 74 IU/L, lactate dehydrogenase 1200 IU/L and serum albumin reduced at 3,4 g/L. Antinuclear antibodies (immunofluorescence-Hep-2 cells), rheumatoid factor (nephelometry), antistreptolysin O, cold agglutinins, cryoglobulins and serum electrophoresis were unremarkable. There was no serological evidence for active or recent infection with the following organisms: Toxoplasma gondii, group B streptococci, hepatitis A, B or C, HIV, cytomegalovirus, tuberculosis and syphilis. Blood cultures were negative. Ultrasound investigation of the liver and biliary tract was normal. In the bone marrow aspiration, we found remarkable hyperplasia and granulocytes of each mature state, without an increase of myeloblasts, the neutrophil alkaline phosphatase score was high, Ph chromose was negative. Immunophenotyping showed mature neutrophils expressing surface antigens CD13 and CD15 at a level higher than 95% and negative for CD34 and HLA-DR.
She was diagnosed with AOSD and methylprednisolone (mini-boluses of 125 mg/ day per 3 days) was initiated as prednisone sparing therapy. The patient's pulmonary symptoms improved with the doses of pulsed corticosteroids. After 3 days of pulsed methylprednisolone, therapy was maintained with 0.5 mg/kg of prednisone orally. Treatment with NSAIDs and prednisone controlled the rash, fever, arthritis, angioedema and significantly reduced the serum ferritin levels and all markers of activity disease. Two weeks after the pulse treatment, the chest radiography became normal. Methotrexate was prescribed for long term control of the disease at a dose of 7,5 mg/week. One month later prednisone was slowly decreased to 12,5 mg/day and withdrawn over three months. No activation period was observed during the one-year follow-up period.
Unusual Paraclinical findings of AOSD in lung. A chest radiograph shows a patchy right lobe consolidation in the lower lung zone (a). CT image shows extensive areas of airspace consolidation due to alveolar hemorrhage in right lung.