BackgroundCollapsing Glomerulopathy (CG), also known as the collapsing variant of Focal Segmental Glomerulosclerosis (FSGS), is distinct in both its clinical severity and its pathophysiologic characteristics from other forms of FSGS. This lesion occurs disproportionally in patients carrying two APOL1 risk alleles, and is the classic histologic lesion resulting from Human Immunodeficiency Virus (HIV) infection of podocytes. Other viral infections, including parvovirus B19, and drugs such as interferon that perturb the immune system, have also been associated with CG. Despite significant advances, explaining such genetic and immune/infectious associations with causative mechanisms and supporting evidence has proven challenging.Case presentationWe report the case of a healthy (HIV-negative) pregnant 36 year-old Caribbean-American woman who presented with nephrotic syndrome and fetal demise in the setting of acute parvovirus B19 infection. A series of three renal biopsies and rapid clinical course showed progression from significant podocyte injury with mild light microscopy findings to classic viral-associated CG to ESRD in less than 3 months. Genetic analysis revealed two APOL1 G1 risk alleles.ConclusionsThis is the first published case report of CG in the setting of acute parvovirus infection in a patient with two APOL1 risk allelles, and parvoviral proteins identified in renal epithelium on kidney biopsy. These findings support the causative role of parvovirus B19 infection in the development of CG on the background of APOL1 genetic risk.Electronic supplementary materialThe online version of this article (doi:10.1186/s12882-016-0330-7) contains supplementary material, which is available to authorized users.
Tumid lupus erythematosus (TLE) is a rare variant of cutaneous lupus erythematosus. Clinically, it lacks typical changes found in discoid lupus and antinuclear antibodies (ANA) levels are elevated in only 10% of the patients. Coexistent systemic lupus erythematosus (SLE) has been reported to be rare, and literature shows only a few case reports. We present a case of coexistent tumid lupus and SLE. We present a case of a 48-year-old Caucasian female who presented with chronic facial rash, photosensitivity, intermittent oral ulcers, joint pain with morning stiffness, and unintentional weight loss. Laboratory studies showed positive ANA at 1:640, elevated erythrocyte sedimentation rate, positive anticardiolipin immunoglobulin (Ig) G, anticardiolipin IgM, and anti-beta-2 glycoprotein IgM. Skin biopsy of the rash showed a superficial and deep dense lymphocytic infiltrate with mucin deposition, histopathology favoring tumid lupus. The patient was diagnosed with TLE with SLE and was started on hydroxychloroquine with improvement in her rash. Ultraviolet light and certain medications have been proven to play a role in the pathogenesis of tumid lupus. It usually responds to photoprotection, topical treatment, or oral antimalarial therapy.
A 65-year-old woman with a medical history of well-controlled scalp psoriasis, chronic kidney disease stage 3, hypothyroidism, and chronic obstructive pulmonary disease was referred to rheumatology for increasing joint pains. The patient had a history of small cell lung cancer (SCLC) with lymph node involvement for 3 years. She had tried and failed carboplatin with etoposide, paclitaxel, topotecan, and chest and cranial radiation therapy. Nivolumab was started for SCLC at the dose of 240 mg every 2 weeks, and after 12 weeks, she started noticing joint pains in her hands, feet, and knees. She presented to the rheumatology office after using From the
Bronchiolitis obliterans organising pneumonia as an initial manifestation of systemic lupus erythematosus (SLE) is a rare and uncommon presentation. We describe a case of SLE presenting with shortness of breath, found to have pneumothorax, bilateral nodular infiltrates along with pleural effusions and pericardial effusion. Work-up suggested a diagnosis of active SLE with anaemia, thrombocytopenia, positive antinuclear antibodies (ANAs) and positive anti-double-stranded DNA. On retrospective review of patient records, from 8 years prior to presentation, lung biopsy histology consistent with bronchiolitis obliterans organising pneumonia with positive ANA serology was found, without any further autoimmune work-up. In our opinion, bronchiolitis obliterans organising pneumonia was the index presentation of SLE. Treatment with steroids and subsequent management with immunosuppressive therapy could have prevented subsequent hospitalisations. Prompt work-up for autoimmune diseases should be considered in patients with positive ANA and histological evidence of bronchiolitis obliterans organising pneumonia.
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