An 82-year-old man was referred for an acute rash predominantly localized to the lower limbs, a 1-week history of symmetrical arthralgia of the wrists and ankles, and recent increasing dyspnoea. His past medical history included a 20-year history of tobacco use, pulmonary tuberculosis 40 years previously, and bladder cancer 25 years previously.
The patient's vital signs were as follows: blood pressure 150/95 mmHg, temperature 37.3 C, heart rate 80/min, respiratory rate 20/min, and percutaneous oxygen saturation 94% without oxygen inhalation. Physical examination revealed a purpuric rash limited to the lower limbs, diffuse wheezing and no obvious arthritis or signs of phlebitis.
Typical biological examinations showed serum electrolytes, creatinine and proteinuria within the normal ranges; C-reactive protein was increased (89 mg/l; normal range <5), haemoglobin was decreased (9.2 g/dl; normal range >13), and platelet count was increased (50x104/mul; normal range <40).
Blood and urine cultures and other serum investigations, including anti-nuclear antibody (ANA), anti-neutrophil cytoplasmic autoantibody (ANCA), rheumatoid factor (RF), anti-cyclic citrullinated peptide antibody (anti-CCP), cryoglobulinemia, HIV and hepatitis B and C, were all negative. Complement factor CH50 was increased, while C3 and C4 were within the normal range. The plasma IgA level was increased (4.29 g/l; normal range 0.70-3.12 g/l). A skin biopsy of the rash showed leukocytoclastic vasculitis (Fig. 1A,B) and perivascular IgA deposits (Fig. 1C).
Chest radiography revealed bilateral pulmonary infiltrates, while a thoracic CT scan showed focal and bilateral parenchymatous infiltrates (Fig. 2).
Brain natriuretic peptide was within the normal range, repeat electrocardiograms remained normal, and transthoracic echocardiograms did not reveal any cardiac dysfunction. Bronchoscopy was normal, while bronchoalveolar lavage (BAL) indicated alveolar haemorrhage with massive haemosiderin-laden macrophages and a Golde score of 258 (normal range <100) with negative direct and culture-based tests for viruses, fungi and bacteria. Based on these data, a diagnosis of IgA vasculitis with cutaneous, articular and pulmonary (intra-alveolar haemorrhage) involvement was made. A few days later, the patient had an episode of macroscopic haematuria, and cystoscopy and a CT scan revealed bladder cancer relapse. However, all clinical signs of IgAV and CT signs of IAH promptly disappeared with administration of methylprednisolone pulses (500 mg/day, 3 days) followed by tapering daily oral prednisone (1 mg/kg/day), and the patient was discharged on day 14 of admission.
During 8 months of follow-up, no IgAV relapses occurred, corticosteroids were stopped in month 3, and the management of relapsing bladder cancer was discussed.