ABSTRACT
BACKGROUND: Systemic scleroderma (SSc) is an insidious autoimmune connective tissue disorder with multiorgan involvement. Renal involvement is one of the important causes of morbidity and mortality in scleroderma; however, nephrotic syndrome is reported rarely in association with SSc. We present a patient with SSc who developed focal segmental glomerulosclerosis (FSGS) as a complication of scleroderma. CASE PRESENTATION: A 59 year old Caucasian female patient, with a known history of diffuse systemic sclerosis from 8 years, presented to our clinic with symptoms of anasarca and weight gain. Her physical examination was unremarkable except for periorbital and extremity edema. Her biochemistry assessment revealed decreased serum albumin levels and elevated serum creatinine levels. A renal biopsy was performed, which showed histopathological patterns of FSGS type of nephrotic syndrome. After administration of high doses of steroid and rituximab in the course of her treatment for 6 months, her symptoms and proteinuria were improved without the occurrence of scleroderma renal crises. CONCLUSION: SSc is a complex multisystemic autoimmune disorder. SRC is the most prominent renal involvement in SSc, but other renal pathologies may also occur. Each patient should be precisely investigated since managing these renal conditions can differ significantly. Nephrotic syndrome is a rare complication of SSc, which could be managed with prompt diagnosis and steroid administration.
Subject(s)
Autoimmune Diseases , Glomerulosclerosis, Focal Segmental , Nephrotic Syndrome , Scleroderma, Localized , Scleroderma, Systemic , Female , Humans , Middle Aged , Nephrotic Syndrome/etiology , Nephrotic Syndrome/complications , Glomerulosclerosis, Focal Segmental/complications , Glomerulosclerosis, Focal Segmental/drug therapy , Kidney/pathology , Proteinuria/etiology , Scleroderma, Systemic/complications , Scleroderma, Systemic/drug therapy , Scleroderma, Localized/complications , Scleroderma, Localized/drug therapy , Steroids/therapeutic useABSTRACT
Key Clinical Message: Clinicians should be aware of rare manifestations of AS, while considering a low threshold for screening vascular involvement in an axial SpA/nrxSpA/AS presenting with unexplained fevers and significant constitutional symptoms and elevated markers. Abstract: Ankylosing spondylitis (AS) is a chronic inflammatory disease from the spondyloarthritis complex, which usually affects young men and primarily involves sacroiliac joints and the spine. It can also present with non-joint involvement, such as cardiovascular manifestations. Aortitis is a rare yet critical cardiovascular complication associated with AS, which can lead to life-threatening outcomes when undiagnosed. Here we report a 34-year-old man with intermittent fevers and significant weight loss, myalgia, and arthralgia for 1 year before being referred to our hospital due to undefinable causes despite multiple diagnostic efforts. The patient presented with elevated inflammatory markers and involvement of sacroiliac joints in favor of the AS. A positron emission tomography scan was also done to rule out underlying malignancy, which led to the detection of inflammation in ascending aorta, compatible with aortitis. The patient was treated with nonsteroidal anti-inflammatory drugs, prednisolone, and infliximab, and his signs and symptoms significantly improved. Our case reports a rare but substantial complication of AS, in a young patient without a history of prolonged disease presenting with unspecific manifestations. The implantation of a thorough examination of AS patients, including cardiac examinations, could contribute to faster and more efficient diagnosis and treatment.
