Juan-Pablo Suso

Catastrophic antiphospholipid syndrome triggered by fulminant Chikungunya infection in a patient with systemic lupus erythematosus

The patient, a 21-year-old woman, was admitted to the intensive care unit with a 4-day history of fever, arthritis of the hands and feet, oli- guria with anasarca, diarrhea, and widespread skin rash. Systemic lupus erythematosus had been diagnosed 5 years previously. She was positive for lupus anticoagulant and anticardiolipin antibody but had no history of thrombotic or obstetric manifestations. Laboratory tests for infection and immunologic or biochemical abnormalities were performed and, based on multiple abnormal findings, treatment with broad-spectrum antibiotics was instituted.

Distinguishing infections vs flares in patients with systemic lupus erythematosus

SLE is a chronic autoimmune disease involving multiple systems. Patients with SLE are highly susceptible to infections due to the combined effects of their immunosuppressive therapy and the abnormalities of the immune system that the disease itself causes, which can increase mortality in these patients. The differentiation of SLE activity and infection in a febrile patient with SLE is extremely difficult. Activity indexes are useful to identify patients with lupus flares but some clinical and biological abnormalities may, however, make it difficult to differentiate flares from infection. Several biological markers are now recognized as potential tools to establish the difference between SLE activity and infection, including CRP and procalcitonin. It is possible, however, that the use of only one biomarker is not sufficient to confirm or discard infection. This means that new scores, which include different biomarkers, might represent a better solution for differentiating these two clinical pictures. This review article describes several markers that are currently used, or have the potential, to differentiate infection from SLE flares.

Is Bariatric Surgery a Trigger Factor for Systemic Autoimmune Diseases

Bariatric procedures are an effective option for weight loss and control of comorbidities in obese patients. Obesity is a proinflammatory condition in which some cytokines such as leptin, a proinflammatory protein, is elevated and adiponectin, an anti-inflammatory protein, is decreased. In patients undergoing weight reduction surgeries, these hormone levels behave paradoxically. It is not known whether bariatric surgery protects against development of autoinflammatory or autoimmune conditions; nevertheless, changes occurring in the immune system are incompletely understood. In this case series, we describe 4 patients undergoing bariatric surgery, who subsequently developed systemic autoimmune diseases. Patients in our case series were asymptomatic before surgery and developed an autoimmune disease within 11.2 months. Two women fulfilled criteria for systemic lupus erythematosus (one associated with antiphospholipid syndrome), and 2 men developed rheumatoid arthritis. A causal relationship is difficult to establish because factors that could trigger these diseases are multiple, including genetic susceptibility, time elapsed until achievement of ideal weight, and vitamin deficiencies, among others. However, clinicians must be attentive to this possible association.

Profile of BAFF and its receptors’ expression in lupus nephritis is associated with pathological classes

Background/Objective B-cell activating factor (BAFF) plays an important role in the pathogenesis of systemic lupus erythematosus. However, the role of BAFF in lupus nephritis (LN) is not understood. Our aim was to evaluate the expression of BAFF and its three receptors in renal biopsy samples from patients with LN and investigate a relationship with pathological class. Methods We conducted a prospective descriptive study (2011–2014) on 52 kidney biopsy samples from patients with LN. Immunohistochemistry for BAFF, its receptors (transmembrane activator and calcium modulator and cyclophilin ligand interaction (TACI), protein maturation of B cells (BCMA), and BAFF-receptor (BAFF-R)), and CD20 expression was performed. Samples were scored according to the percentage of cells with positive expression. Results In class II LN, BAFF-R and TACI were not expressed, whereas BCMA and BAFF were lowly expressed in the interstitial inflammatory infiltrates. Proliferative class III/IV had elevated BAFF expression in the glomeruli, and TACI was expressed in interstitial inflammatory infiltrates and the glomeruli. Interestingly, the class IV cases with vasculopathy (n = 4) had endothelial BAFF expression, which was not visible in thrombotic microangiopathy (n = 4). Class V was characterized by low BAFF expression in interstitial inflammatory infiltrates and by BAFF, TACI, and BCMA expression in the glomeruli. BAFF expression was associated with inflammatory scores and CD20 positive infiltrates, mainly in class IV. Conclusions Expression patterns of BAFF and its receptors differ according to LN class. Our study provides evidence that BAFF could be used as a routine marker in LN biopsies and to determine which patients will benefit from anti-BAFF therapy.

Severe deformans tophaceous gouty arthritis.

A 61-year-old man with a 20-year history of gout with severe functional disability was evaluated in our service. He received alopurinol and colchicine without response. Physical examination revealed bilateral great deformity, multiple tophi and edema of hands (Fig. 1), feet and knees. Serum uric acid was elevated: 11.2 mg/dL (reference value: 3.4–7.0 mg/dL). Hands X-ray AP view demonstrated, in both hands (Fig. 2A [left] and B [right]), conserved bone mineralization with severe soft-tissue swelling, extensive erosions with sclerotic borders (white arrows), precipitation of calcium with urate crystals giving density to the tophi (head arrows), bone spiculations (black arrows) and osteolytic expansive lesions in multiple heads of metacarpophalangic joint (double-head arrows).