Lymphadenopathy as a Prodrome for Systemic Lupus Erythematous.

BACKGROUND: Lupus lymphadenopahy (LL) has an estimated incidence of 1% at diagnosis. Here, we report a case of systemic lupus erythematous which presented with a prodrome of generalized lymphadenopathy and fevers. CASE PRESENTATION: A 41-year-old woman presented to the hospital with one month of fevers, chills, lymphadenopathy, abdominal pain, a bilateral upper extremity rash, and malaise. Physical exam was notable for tender, palpable posterior cervical lymph nodes that were mobile and about 1 cm in maximum diameter. After extensive infectious, hematologic, and autoimmune evaluations, a diagnosis of systemic lupus erythematous (SLE) was made and treatment with high-dose steroids and hydroxychloroquine which resulted in gradual improvement in symptoms. CONCLUSION: Systemic lupus erythematous can present with a subtle prodrome of generalized lymphadenopathy. It is important for medical professionals to consider SLE in the differential in a patient with diffuse lymphadenopathy.

Tongue Stiffness as Presentation of Antiphospholipid Syndrome: A Case Report.

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder with marked thrombotic and inflammatory features driven by the presence of antiphospholipid antibodies (APLA). Here, we report a case of APS with a rare, atypical manifestation and discuss a differential diagnosis. A 53-year-old male without significant past medical history presented with new onset of episodic tongue stiffness and dysarthria which lasted for about a minute over a period of three months. This was associated with intermittent right retro-orbital sharp pain radiating to the parietal area. He also reported swelling and stiffness of the third and fourth right proximal interphalangeal (PIP) joints lasting throughout the day. A physical exam revealed tongue fasciculations. As the MRI showed patchy white matter hyperintensities neurology initially suspected multiple sclerosis. However, cerebrospinal fluid (CSF) analysis including neuromyelitis optica (NMO) antibodies and oligoclonal antibodies was negative. Rheumatological work up was remarkable for positive antinuclear antibodies (ANA); anticardiolipin antibodies and lupus anticoagulant were positive 12 weeks apart. This, alongside with stable white matter changes on imaging was suspicious for an extra-criteria manifestation of antiphospholipid antibody syndrome. The most commonly described neurological manifestations of APS are headache, transient ischemic attack (TIA), and stroke. Tongue stiffness as an initial symptom is quite unusual and, to the best of our knowledge has not been reported in medical literature. In patients with isolated neurological findings of unclear etiology, an autoimmune disease such as APS should be considered, and appropriate diagnostic work up should not be postponed. Unfortunately, positive laboratory markers can have a wide differential diagnostic panel. In addition, APS may mimic many diseases both in clinical presentation and MRI findings thus making the correct diagnosis challenging. However, studies show that, unlike multiple sclerosis (MS), white matter changes in APS remain static during the course of the disease. Identification of atypical presentations of APS is critical as prompt and correct medical management can improve patients' quality of life and clinical outcomes.

'Carpal tunnel syndrome' and 'tennis elbow' as prodromes for granulomatosis with polyangiitis (formerly Wegener's granulomatosis).

A 62-year-old man presented with excruciating joint pains, back stiffness and numbness of his hands and feet. Over the past 18 months, he had experienced similar episodes for which the diagnoses of bilateral carpal tunnel syndrome and lateral epicondylitis had been made. Physical examination revealed polyarticular arthritis affecting the shoulders, wrists and right knee. Palpable purpura overlying the calves and ankles was present. Laboratory tests showed markedly elevated erythrocyte sedimentation rate and C-reactive protein in the setting of negative blood and urine cultures. Rheumatoid factor and antinuclear antibodies were negative. Chest CT demonstrated bilateral pulmonary infiltrates. A punch biopsy of the rash showed leukocytoclastic vasculitis. Anti-proteinase-3 titers returned strongly positive. A diagnosis of granulomatosis with polyangiitis was made. Treatment with high-dose steroids, followed by rituximab resulted in normalisation of inflammatory markers with subsequent resolution of joint pains, rash and pulmonary infiltrates and improvement of neuropathic symptoms.