Kikuchi-Fujimoto Disease With Systemic Lupus Erythematosus and Systemic Sclerosis Overlap: A Unique Clinical Presentation.

Kikuchi-Fujimoto Disease (KFD), or histiocytic necrotizing lymphadenitis (HNL), is a rare self-limiting disorder presenting with fever and swollen lymph nodes. It is characterized by the focal proliferation of reticular cells, the presence of nuclear debris, and histiocytes. In advanced cases, it can present with hepato-splenomegaly and generalized lymphadenopathy. Historically, it has been associated with viral infections, as it frequently was found to be associated with upper respiratory symptoms. Alternative explanations include the immune response of T-cells leading to alteration in CD8-positive T-cell-mediated cell apoptosis. It is also speculated that KFD can be associated with rheumatological autoimmune diseases. We present a case of a 21-year-old African American female with a known diagnosis of systemic lupus erythematosus (SLE)-systemic sclerosis (SS) overlap presented with febrile lymphadenopathy and was diagnosed to have HNL on histological exam of lymph node biopsy.

Hydralazine-Induced Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Asymptomatic and Renal-Restricted Presentation.

BACKGROUND Hydralazine, a potent vasodilator widely used to treat hypertension, has been implicated in an increasing number of cases of drug-induced autoimmune diseases in recent years. However, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis secondary to hydralazine use has rarely been described and most reported cases involved multi-organ-related vasculitis, including skin and lung-kidney manifestations. ANCA-associated vasculitis is an immune-inflammatory condition characterized by necrotizing vasculitis with few or no immune deposits, predominantly affecting small vessels. The fact that the vasculitis is associated with hydralazine use and improves with discontinuation of hydralazine supports the diagnosis of hydralazine-induced disease. The case we report is a hydralazine-induced, ANCA-associated, pauci-immune crescentic glomerulonephritis with a presentation limited to the kidneys. CASE REPORT A 66-year-old woman was admitted to the hospital for worsening renal function over a month with no symptoms. Serology work-up was significantly positive for antinuclear, perinuclear ANCA, anti-histone, anti-double-stranded DNA, anti-cardiolipin, and anti-myeloperoxidase antibodies. The patient ultimately underwent a kidney biopsy, which revealed pauci-immune crescentic glomerulonephritis. Her kidney function improved with cessation of hydralazine as well as therapy with pulse steroids. CONCLUSIONS Hydralazine is commonly prescribed to treat hypertension. Healthcare providers should be aware of potentially severe hydralazine-induced ANCA-associated vasculitis, which can present with various clinical manifestations. Serologic studies have indicated that it has features that overlap with lupus. Biopsy is helpful for making a definitive diagnosis and developing individual treatment plans. Early diagnosis, cessation of the offending drug, and initiation of immunosuppressive therapy are key for favorable prognosis.

Macrophage Activation Syndrome Upon Initiation of Adalimumab in a Patient With Longstanding Rheumatoid Arthritis.

Macrophage activation syndrome (MAS) is a subset of hemophagocytic lymphohistiocytosis (HLH) described in patients with rheumatological disorders. Some triggers of MAS and HLH include infection, malignancy, rheumatological disease, HIV, and rarely medications such as immunosuppressants. In recent medical literature, biologic agents are increasingly recognized as a potential trigger, but the mechanism behind this remains poorly understood. We describe the case of a patient who developed MAS after initiating adalimumab and propose a potential pathophysiological link between biologics and this syndrome.

Rheumatoid Arthritis as a Therapeutic Challenge in a Patient with Lynch Syndrome.

BACKGROUND: Lynch syndrome (LS) is an inherited colorectal cancer (CRC) syndrome accounting for about 3-5% of all cases and involves significantly higher risk of subsequent malignancies, colonic as well as extra-colonic. Increased risk of malignancies, especially lymphoid malignancies, have been described in patients with autoimmune diseases like rheumatoid arthritis (RA), systemic lupus erythematosus, and Sjögren's syndrome. Epidemiological studies demonstrated that hematopoietic, lung, skin, and prostate cancers are increased in RA, while breast and colon cancers are decreased, with an overall slight increase in all cancers. CASE REPORT: Our case demonstrates the development of CRC, endometrial cancer, and breast cancer as a presentation of LS in a patient with RA and presents a therapeutic challenge for RA treatment. CONCLUSIONS: We describe a patient with LS and RA presenting a therapeutic challenge because biologic agents commonly used to treat severe RA need to be used cautiously in patients with history of malignancy.

A Rare Case of Neuromyelitis Optica Spectrum Disorder in Patient with Sjogren's Syndrome.

We report a 48-year-old female with the history of Sjogren's syndrome who presented with 3-week history of tingling, numbness, and shooting back, waist, and bilateral leg pain and numbness in the pelvic region with urinary and bowel incontinence. Physical examination was remarkable for reduced motor power in both lower extremities with spasticity. Sensory deficit was noted at the T6 level. Laboratory investigation revealed elevated ESR and CRP and positive serum antiaquaporin-4 IgG. Thoracic and lumbar magnetic resonance imaging revealed abnormal patchy areas, leptomeningeal enhancement through the thoracic cord extending from T3 through T6 levels, without evidence of cord compression. Impression of neuromyelitis optica spectrum disorder was made and patient was treated with methylprednisolone intravenously followed by tapering oral prednisone. Neurological symptoms gradually improved with resolution of bowel and urinary incontinence. In a patient with Sjogren's syndrome who presents with neurological complaints, the possibility of neuromyelitis optica or neuromyelitis optica spectrum disorder should be considered. Awareness of the possibility of CNS disease is important due to the serious nature of CNS complications, some of which are treatable with immunosuppressants. Our patient with Sjogren's syndrome who presented with myelopathy benefited from early recognition and institution of appropriate therapy.

Interstitial granulomatous dermatitis successfully treated with etanercept.

PATIENT: Female, 51 FINAL DIAGNOSIS: Interstitial Granulomatous Dermatitis Symptoms: Joint pain • pruritic rush MEDICATION: Etanercept Clinical Procedure: - Specialty: Rheumatology. OBJECTIVE: Rare disease. BACKGROUND: Interstitial granulomatous disease (IGD) is a rare skin condition that presents with erythematous and violaceous plaques, and may be associated with pruritus and pain. The cause remains unknown, but is often associated with autoimmune disease and drug-related adverse effects. It is diagnosed via biopsy, and the treatment remains unclear. CASE REPORT: We report a case of biopsy-proven IGD associated with rheumatoid arthritis that was treated successfully with etanercept therapy. CONCLUSIONS: We emphasize that anti-TNF antibodies may be clinically effective for the treatment of IGD.

[Vitamin D levels in ankylosing spondylitis: does deficiency correspond to disease activity?]. / Níveis de vitamina D na espondilite anquilosante: a deficiência corresponde à atividade da doença?

Ankylosing spondylitis (AS) is an inflammatory disorder that presents with arthritis of the axial skeleton, including sacroiliac joints. Vitamin D is a secosteroid hormone with a long-established role in calcium and phosphate homeostasis, and in the regulation of bone formation and resorption. It is now known that vitamin D plays an immunosuppressive role in the body, and there is interest of late in the role of vitamin D in autoimmune diseases. Inflammation may be responsible for some of the loss of bone mineral density seen in AS. We reviewed the literature for studies assessing vitamin D level as a marker of AS disease activity and those examining vitamin D levels in AS in comparison to healthy controls. Four of 7 studies found a significant negative correlation between vitamin D levels and Bath Ankylosing Spondylitis Index (BASDAI), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). In a review of 8 case-control studies, the mean level of 25-hydroxyvitamin D3 was 22.8 ± 14.1 ng/mL in 555 AS patients versus 26.6 ± 12.5 ng/mL in 557 healthy controls. When compared with a 2-sample t test, vitamin D levels were significantly higher in healthy controls (p < 0.01). We conclude that patients with AS appear to have lower vitamin D levels versus healthy controls; however, the cause is unclear. Existing studies do not demonstrate a consistent link between vitamin D levels and disease activity in AS. Further studies are in need to determine if a causative link exists between vitamin D deficiency and AS.

Adalimumab-induced acute pneumonitis in a patient with rheumatoid arthritis.

Interstitial lung disease is one of the most common and severe extra-articular manifestations associated with rheumatoid arthritis. Previous to the biologic treatment era, methotrexate was the medication known to cause acute lung disease mostly in patients with preexisting rheumatoid lung disease. However, recent case reports of patients treated with biologic therapies show an increased incidence of acute lung disease caused by tumor necrosis factor alpha inhibitors. This case will illustrate acute lung disease caused by adalimumab, a recombinant IgG1 monoclonal antibody. The rheumatology community must be aware of this adverse effect described so far with all 3 major tumor necrosis factor alpha inhibitors, before starting but also during maintenance therapy.