An unexpected case of cryoglobulinaemia vasculitis that presented as respiratory failure

Cryoglobulinaemia vasculitis is a type of small vessel vasculitis caused by deposition of immune complexes with associated inflammation of the blood vessels. We report a case of severe manifestation of cryoglobulinaemic vasculitis with renal involvement and alveolar haemorrhages. A 66-year-old lady presented with a widespread petechial rash associated with high grade fever, sore throat, and arthralgia. The rash was distributed over her elbows, legs, and ankles. There was history of unintentional weight loss. Her past medical history included Type II diabetes mellitus. More recently, she required a course of steroids for leukoclastic vasculitis. On admission, she was in septic shock that required fluid resuscitation and intravenous antibiotics. Initial investigations revealed raised inflammatory markers alongside elevated D-Dimer of 5,140 and pro BNP of 8,323. The chest X-ray demonstrated right sided opacification. She was initially treated as sepsis secondary to community acquired pneumonia. A full septic screen was sent. Furthermore, autoimmune screen was requested due to concerns about vasculitis rash. Rheumatology advice was sought and subsequently she was started on a low dose prednisolone 20 mg. A CT pulmonary Angiogram was performed that ruled out a pulmonary embolism but confirmed bilateral pleural effusions. An Echocardiogram was essentially normal. She developed acute respiratory deterioration and generalised fluid overload, requiring high flow nasal oxygen and furosemide infusion. The blood tests showed worsening renal function and hypalbuminaemia. Furthermore, a repeat CT Chest revealed widespread ground glass opacifications in keeping with alveolar haemorrhages. Subsequently, the autoimmune panel showed low C4 complement levels with normal C3. The cryoglobulinaemia screen was positive for Type II IgM cryoglobulinaemia. Due to respiratory deterioration and renal function decline, she was transferred to intensive care unit and required intubation and ventilation. The case was discussed with the tertiary centre with plan for transfer for plasma exchange and Rituximab. Unfortunately, she went into multiorgan failure and passed away before receiving further treatment. Cryoglobulinaemia vasculitis is an immune mediated inflammation of small and medium size blood vessels secondary to deposition of abnormal proteins called cryoglobulin. The mechanism of cryoprecipitation is complex and is attributed by precipitation of cryoglobulins when serum temperature falls below 37 °C. These immunoglobulins aggregate within the blood vessel and activate immune mediated pathways which contribute to inflammation. Cryoglobulinaemia vasculitis usually occurs secondary to other disease. Cryoglobulinaemia is classified into three groups depending on the type of Immunoglobulins. The management of Cryoglobulinaemia vasculitis is guided by the underlying aetiology of disease, the severity of condition and associated organ involvement. Furthermore, immunosuppression with Rituximab or cyclophosphamide reduces risk of relapse. The case represents severe manifestations of cryoglobulinaemia vasculitis with associated pulmonary haemorrhages and possible progressive glomerulonephritis. Unfortunately, further management with plasmapheresis and Rituximab could not be commenced due to rapid deterioration. Cryoglobulinaemia vasculitis is a debilitating condition with recurrent relapses and high mortality rate. It is also associated with increased risk of complications and requires multidisciplinary team approach to manage the condition effectively.