Clinical characteristics, radiological features and outcomes in pulmonary involvement of cryoglobulinemia.

BACKGROUND: Cryoglobulinemia with pulmonary involvement is rare, and its characteristics, radiological findings, and outcomes are still poorly understood. METHODS: Ten patients with pulmonary involvement of 491 cryoglobulinemia patients at Peking Union Medical College Hospital were enrolled in this retrospective study. We analyzed the characteristics, radiological features and management of pulmonary involvement patients, and compared with those of non-pulmonary involvement with cryoglobulinemia. RESULTS: The 10 patients with pulmonary involvement (2 males; median age, 53 years) included three patients with type I cryoglobulinemia and seven patients with mixed cryoglobulinemia. All of 10 patients were IgM isotype cryoglobulinemia. All type I patients were secondary to B-cell non-Hodgkin lymphoma. Four mixed patients were essential, and the remaining patients were secondary to infections (n = 2) and systemic lupus erythematosus (n = 1), respectively. Six patients had additional affected organs, including skin (60%), kidney (50%), peripheral nerves (30%), joints (20%), and heart (20%). The pulmonary symptoms included dyspnea (50%), dry cough (30%), chest tightness (30%), and hemoptysis (10%). Chest computed tomography (CT) showed diffuse ground-glass opacity (80%), nodules (40%), pleural effusions (30%), and reticulation (20%). Two patients experienced life-threatening diffuse alveolar hemorrhage. Five patients received corticosteroid-based regimens, and four received rituximab-based regimens. All patients on rituximab-based regimens achieved clinical remission. The estimated two-year overall survival (OS) was 40%. Patients with pulmonary involvement had significantly worse OS and progression-free survival than non-pulmonary involvement patients of cryoglobulinemia (P < 0.0001). CONCLUSIONS: A diagnosis of pulmonary involvement should be highly suspected for patients with cryoglobulinemia and chest CT-indicated infiltrates without other explanations. Patients with pulmonary involvement had a poor prognosis. Rituximab-based treatment may improve the outcome.

A 47-Year-Old Woman With Progressive Exertional Dyspnea and Fatigue.

CASE PRESENTATION: A previously healthy 47-year-old nonsmoking woman was admitted to our hospital with an 8-month history of progressive exertional dyspnea and fatigue. Chest high-resolution CT (HRCT) on admission showed diffuse, bilateral, patchy ground-glass opacity (GGO) (Fig 1A). She was diagnosed with interstitial lung disease, and corticosteroid therapy with 8 weeks prednisone taper was completed, with initial good response. Eight months later, she was readmitted because of worsening of the dyspnea, with no fever, wheeze, dry cough, chest pain, weight loss, or hemoptysis. She denied a history of hair loss, skin rash, oral ulcers, or arthralgia. She denied a history of allergy or taking other drugs. She had no occupational or environmental exposures. There was no family history of respiratory diseases or hematologic diseases.

Neurologic manifestations associated with cryoglobulinemia: A single center experience.

INTRODUCTION: Limited information is available describing the spectrum of neurological complications of cryoglobulinemia. METHODS: Single center retrospective review of patients with neurologic symptoms and elevated serum cryoglobulins, with their potential association being classified as definite, possible, or unlikely using defined criteria. RESULTS: Among 492 patients, 131 (87 classified as definite and 44 as possible) had neurologic symptoms associated with cryoglobulinemia. Common comorbidities included hepatitis C (N = 43), monoclonal gammopathy of undetermined significance (N = 20), Sjogren's syndrome (N = 17), membranoproliferative glomerulonephritis (N = 17), and systemic lupus erythematosus (N = 10). Features supporting an association between cryoglobulinemia and neurological symptoms were the presence of purpura (p < .001), positive rheumatoid factor (p = .001) and low C4 (p = .002). Common peripheral neurological diagnoses were symmetric polyneuropathy (N = 84), small fiber neuropathy (N = 25), and mononeuritis multiplex (N = 16). Central neurological manifestations were infrequent and included seizures (N = 3), posterior reversible encephalopathy syndrome (N = 2), intracerebral hemorrhage (N = 1), vasculitis (N = 1), rapidly progressive dementia (N = 1), lymphoma (N = 1), and myelitis/meningitis (N = 1). Treatments utilized included corticosteroids (N = 74), rituximab (N = 42), cyclophosphamide (N = 27), methotrexate, azathioprine, or mycophenolate mofetil (N = 28), anti-viral therapy (N = 20), plasmapheresis (N = 16), and intravenous immunoglobulin (N = 20). Neurologic symptoms associated with cryoglobulinemia remained stable or improved in 86% of patients. CONCLUSION: This study describes a wide spectrum of patients with neurologic symptoms attributed to cryoglobulinemia and provides a framework to approach this challenging diagnosis.

Cryoglobulinaemic glomerulonephritis: artefacts associated with laboratory diagnosis and need for renal biopsy.

Cryoglobulinemia as a cause of renal impairment is uncommon but needs to be considered in viral hepatitis and haematological malignancies. Often detection and estimation of cryoglobulins are confounded by collection and processing errors. This report highlights the need for stringent processing measures if the clinical suspicion is high.

Laminar crystal deposition in large vessels in a patient with crystalglobulinemia.

Crystalglobulinemia is an extremely rare complication of monoclonal gammopathy and is characterized by crystal thrombi within systemic organs. We herein report the first described case of crystalglobulinemia accompanied by laminar crystal deposition in the large vessels. A 44-year-old man presented with a history of numbness, pain, and swelling of the left leg in addition to visual impairment. Renal and skin biopsies revealed crystal thrombi within the capillary lumens. The patient was finally diagnosed with crystalglobulinemia associated with multiple myeloma. He was treated with hemodialysis and chemotherapy but died of the disease 15 months after admission. Autopsy demonstrated a huge amount of crystal deposition in the subintimal layer of the vascular wall throughout the thoracic to abdominal aorta. The characteristic deposition extended to the iliac arteries, common carotid arteries, and subclavian arteries but did not affect the bilateral renal arteries. Antemortem computed tomography demonstrated higher intensity in the wall of the abdominal aorta but not in the walls of the renal arteries, suggesting that a finding of high intensity on computed tomography could be a clinical marker of systemic crystal deposition.

Intraosseous pneumatocysts: a case based review.

Intraosseous pneumatocysts are benign gaseous bone lesions, generally asymptomatic and incidentally discovered on X-ray. These characteristics explain why these lesions are unknown to most practitioners and their low representation in medical literature. The description of an incidentally discovered iliac pneumatocyst gives us the opportunity to provide a review of available medical literature on these frequent, but poorly known lesions.

[Symmetrical peripheral gangrene of unknown etiology]. / Symmetrische periphere Gangrän unbekannter Atiologie.

We describe the case of a 19-year-old female patient who presented with acute symmetrical peripheral gangrene (SPG), including all fingers and toes. Further diagnostic work-up did not reveal any underlying condition other than a non-specific transient cryoglobulinemia. Despite immediate anticoagulopathic and anti-inflammatory treatment, all fingers and toes had to be amputated.

Case report: the need for caution when using the spleen to assess renal blood flow in renography.

Visual assessment of renal blood flow from renography is usually achieved by comparing the count rate from the kidney with that of the spleen during the first pass of the radiopharmaceutical agent. This is not justifiable if splenic blood flow is abnormal. In nine subjects without evidence of renal or other significant disease, the mean ratio of the slopes of the first pass curves over the left kidney and the spleen was 1.7 (SD 0.32). However, an additional patient, a 67-year-old female with type-II cryoglobulinaemia, illustrated the need for caution when using this approach. Her splenic blood flow was significantly elevated, resulting in a kidney/spleen slope ratio of only 0.66, even though her renal function was thought to be normal.