Unusual Presentation of Relapsing Polychondritis in a Patient with Human Immunodeficiency Virus and Reactive Arthritis.

BACKGROUND Relapsing polychondritis (RP) is an uncommon autoimmune condition that impacts cartilaginous structures involving the ears, nose, respiratory tract, and joints. Its etiology is unknown; however, it may be associated with other systemic autoimmune diseases, malignancy, and rarely with human immunodeficiency virus (HIV) infection. RP has a variable pattern at presentation and may be associated with constitutional symptoms such as fever and arthralgia, in addition to various auricular, ocular, respiratory, and cardiovascular manifestations. Auricular and ocular signs are the most common presenting features; however, idiopathic orbital inflammatory syndrome is considered a rare manifestation of the disease. Systemic corticosteroids are the mainstay of treatment, but immunomodulatory therapy may be required for refractory cases. CASE REPORT We present a challenging case of RP in a 58-year-old woman with HIV controlled by highly active anti-retroviral therapy (HAART) and stable chronic reactive arthritis on sulfasalazine who developed unilateral auricular chondritis associated with contralateral idiopathic orbital inflammation and scleritis as well as worsening arthralgia. She was initially treated empirically with antibiotics, without clinical improvement. The infectious diseases work-up was unrevealing, and other diagnostic possibilities were meticulously excluded. Clinical suspicion for RP ultimately led to appropriate therapy with corticosteroids and subsequent immunosuppression with methotrexate, resulting in clinical improvement and allowing for gradual tapering of steroids. CONCLUSIONS RP is an uncommon multisystem disorder that can occur in the setting of other underlying chronic illnesses, as seen in our patient. It has a variable presentation and course, with no diagnostic laboratory tests; therefore, clinical suspicion is imperative for appropriate diagnosis and management.

Propylthiouracil-Induced Anti-Neutrophil Cytoplasmic Antibody Vasculitis Presenting with Red Eye Followed by Pulmonary Hemorrhage: Diagnostic and Management Considerations.

BACKGROUND Drug-induced anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) should be suspected in patients on certain medications who present with inflammatory ocular, constitutional, pulmonary, and/or renal manifestations. Here, we present a case of propylthiouracil (PTU)-induced AAV presenting initially with red eye, and review important diagnostic and management considerations for this uncommon disorder. CASE REPORT A 34-year-old woman with hyperthyroidism taking PTU presented with red eye, later followed by fevers and hemoptysis. She was found to have episcleritis, diffuse alveolar hemorrhage, and microhematuria. The infectious diseases workup was unrevealing. Laboratory evaluations were notable for a high-titer perinuclear ANCA and elevated anti-myeloperoxidase antibodies. Renal function was normal. She was ultimately diagnosed with PTU-induced AAV. PTU was promptly discontinued and she was treated with pulse-dose methylprednisolone for 3 days, followed by prednisone 60 mg daily. A kidney biopsy revealed pauci-immune focal segmental necrotizing and crescentic glomerulonephritis. Given an allergy to methimazole, she underwent thyroidectomy and was ultimately treated with rituximab. Her steroid doses are progressively being tapered and she has complete resolution of symptoms. CONCLUSIONS PTU-induced AAV is a rare and serious condition. Our patient presented with ocular symptoms prior to more commonly recognized pulmonary and renal manifestations. Patients may have favorable outcomes if PTU is discontinued promptly, but patients with vital-organ involvement may require treatment with steroids and may need additional immunosuppression.

Longitudinal extensive transverse myelitis: a rare neurological complication of systemic lupus erythematosus.

We present a 47-year-old woman with recently diagnosed systemic lupus erythematosus who developed progressive numbness and tingling of her upper and lower extremities, followed by weakness and difficulty ambulating. She was diagnosed with longitudinal extensive transverse myelitis involving her entire cervical and thoracic spinal cord. Infectious workup was unrevealing. She failed to respond to pulse-dose intravenous steroids, but slowly improved with the addition of plasmapheresis and cyclophosphamide. Following maintenance treatment with mycophenolate mofetil and slow tapering of oral steroids, she has maintained complete remission with significant recovery of neurological function.

Persistent cervical lymphadenitis in a patient with prior thyroid cancer attributed to Kikuchi-Fujimoto disease.

We describe a 50-year-old woman with a history of thyroid cancer who presented with bilateral cervical and submandibular lymphadenopathy, low-grade fevers, and increasing fatigue. The patient underwent lymph node fine-needle aspiration, which showed no evidence of metastatic or lymphoproliferative disease. This procedure was complicated by a parapharyngeal abscess and cellulitis. She was treated unsuccessfully with various courses of antibiotics, but briefly responded to short courses of steroids. As her cervical lymphadenopathy returned, she underwent an excisional lymph node biopsy, which demonstrated caseating granulomatous lymphadenitis. Extensive review of systems, physical examination, laboratory and imaging studies demonstrated no evidence of malignancy, infection or systemic lupus erythematosus . Our patient was clinically diagnosed with Kikuchi-Fujimoto disease and successfully treated with prednisone tapered over 3 months. She remains in clinical remission.

A tale of two vasculitides: biopsy-proven giant cell arteritis followed by the independent development of renal-limited microscopic polyangiitis.

We describe an 80-year-old woman who presented with headaches, bilateral jaw claudication and visual symptoms. She was diagnosed with giant cell arteritis, which was confirmed by temporal artery biopsy. She was treated with high-dose prednisone followed by a slow taper, with complete resolution of symptoms. Approximately 4 years later, she developed progressively worsening renal function associated with haematuria. Serological workup revealed a high-titre perinuclear antinuclear cytoplasmic antibody and antibodies to myeloperoxidase. Renal biopsy demonstrated a pauci-immune focal crescentic glomerulonephritis. Extensive review of systems, physical exam and diagnostic studies demonstrated no evidence of extra-renal disease, and she was diagnosed with renal-limited microscopic polyangiitis. High-dose prednisone was resumed, but she refused treatment with either cyclophosphamide or rituximab due to concern for toxicity. Her prednisone dose was tapered and renal function stabilised. Our case highlights the need to recognise the successive occurrence of two distinct vasculitides in a single patient and monitor accordingly.

Refractory postsurgical pyoderma gangrenosum in a patient with Beckwith Wiedemann syndrome: response to multimodal therapy.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that may be difficult to diagnose and treat. We presented a 41-year-old woman who required skin grafting following third-degree burns to her left breast. She suffered recurrent graft dehiscence and infections over many years, prompting elective bilateral reduction mammoplasty. She subsequently developed suture margin ulcerations unresponsive to topical therapies and antibiotics. Skin biopsies were non-specific, and a clinical diagnosis of PG was established. Although initially responsive to corticosteroids, wounds promptly recurred following steroid taper. She was treated unsuccessfully with various immunomodulatory agents and underwent elective bilateral mastectomy. Following a mastectomy, she developed progressive deep chest wall ulcerations. She failed numerous immunomodulatory treatments, surgical wound closure and negative pressure wound therapy. Ultimately, treatment with adalimumab, mycophenolate mofetil and prednisone, in addition to hyperbaric oxygen therapy facilitated progressive healing. Our case highlights the role of collaborative multimodal therapy for the treatment of refractory PG.

Idiopathic retroperitoneal fibrosis presenting with recurrent bilateral uveitis.

Patients with recurrent uveitis are often evaluated for the presence of underlying systemic disease. The authors describe a 55-year-old black female who presented with isolated recurrent anterior uveitis. Laboratory evaluations were notable for elevated inflammatory markers. She subsequently developed left lower extremity painless swelling; ultrasound evaluation was negative for deep venous thrombosis. CT scan of her abdomen and pelvis demonstrated multiple amorphous soft tissue densities throughout the small bowel mesentery and retroperitoneum, associated with left-sided hydronephrosis. Histopathology of a retroperitoneal mass demonstrated retroperitoneal fibrosis (RPF). Treatment with systemic corticosteroids led to shrinkage of her mesenteric and retroperitoneal masses, resolution of uveitis and normalisation of inflammatory markers. Albeit rare, RPF should be considered in the diagnostic investigation of patients with recurrent uveitis, especially those with abdominopelvic or lower extremity complaints.

Relationship of asymmetric dimethylarginine and homocysteine to vascular aging in systemic lupus erythematosus patients.

OBJECTIVE: Systemic lupus erythematosus (SLE) is independently associated with accelerated atherosclerosis and premature arterial stiffening. Asymmetric dimethylarginine (ADMA) and homocysteine are mechanistically interrelated mediators of endothelial dysfunction and correlates of atherosclerosis in the general population. The aim of this study was to assess the relationship of ADMA and homocysteine to subclinical vascular disease in patients with SLE. METHODS: One hundred twenty-five patients with SLE who were participating in a study of cardiovascular disease underwent clinical and laboratory assessment, carotid artery ultrasonography to detect atherosclerosis, and radial artery applanation tonometry to measure arterial stiffness. RESULTS: Neither ADMA nor homocysteine correlated with the presence or extent of carotid atherosclerosis. In contrast, ADMA was significantly related to the arterial stiffness index. Independent correlates of arterial stiffening included the ADMA concentration, the presence of diabetes mellitus, older age at the time of diagnosis, longer disease duration, and the absence of anti-Sm or anti-RNP antibodies. A secondary multivariable analysis substituting homocysteine for ADMA demonstrated comparable relationships with arterial stiffness (r(2) = 0.616 for homocysteine and r(2) = 0.595 for ADMA). CONCLUSION: ADMA and homocysteine are biomarkers for and may be mediators of premature arterial stiffening in patients with SLE. Because arterial stiffness has independent prognostic value for cardiovascular morbidity and mortality, its predictors may identify patients who are at increased risk of cardiovascular disease.