Ankylosing Spondylitis and Cardiac Manifestations

Ankylosing spondylitis (AS) is a systemic disorder that affects multiple systems in the body. One of the extra-articular manifestations of AS is cardiac involvement, which can lead to heart block and aortic regurgitation. Chronic aortic regurgitation can cause left ventricular hypertrophy, resulting in deep S in V1 and tall R in V5. A-V dissociation is the ECG manifestation of heart block. Mortality rates are higher in patients with AS, with circulatory disease being the most common cause of death.

P-pulmonale is not a typical manifestation of AS, but it can occur in cases of pulmonary hypertension when the right atrium is enlarged. Pulmonary fibrosis, which can occur in AS, can theoretically lead to pulmonary hypertension and p-pulmonale. Right bundle branch block is a non-specific ECG finding that can occur without any heart disease or in conditions such as atrial septal defect, ischemic heart disease, or pulmonary embolism.

In conclusion, AS is a systemic disorder that can affect multiple systems in the body, including the heart. Cardiac involvement can lead to heart block and aortic regurgitation, which can cause left ventricular hypertrophy. Mortality rates are higher in patients with AS, with circulatory disease being the most common cause of death. While p-pulmonale is not a typical manifestation of AS, it can occur in cases of pulmonary hypertension. Right bundle branch block is a non-specific ECG finding that can occur in various conditions.

Diagnostic Markers for Polymyalgia Rheumatica and Temporal arthritis

Polymyalgia rheumatica and temporal arthritis are inflammatory conditions that can cause significant morbidity if left untreated. Here are some diagnostic markers that can help support or rule out these conditions:

Plasma viscosity: A raised plasma viscosity can support a diagnosis of polymyalgia rheumatica with temporal arthritis, but it is a nonspecific inflammatory marker.

Creatine kinase: A raised creatine kinase is not supportive of a diagnosis of polymyalgia rheumatica or temporal arthritis.

Monospot test: A positive monospot test is supportive of a diagnosis of Epstein–Barr virus (EBV), but not polymyalgia rheumatica or temporal arthritis.

Whole cell count (WCC): A raised WCC is not supportive of a diagnosis of polymyalgia rheumatica or temporal arthritis.

Bence Jones proteins: Presence of Bence Jones protein is supportive of a diagnosis of multiple myeloma, but not polymyalgia rheumatica or temporal arthritis.

If temporal arthritis is suspected, immediate treatment with prednisolone is crucial to prevent permanent loss of vision. A temporal artery biopsy can confirm the diagnosis.

When it comes to bone density, T scores are an important measure to understand. A T score of less than -2.5 is indicative of osteoporosis, while a T score between -1 and -2.5 suggests osteopenia. On the other hand, a T score of 0-1 is considered normal, but may still require monitoring. A T score greater than 2.5 is also normal, but may not be the case if the patient has experienced a fragility fracture. It’s important to note that Z scores, which take into account age and gender, can also provide insight into bone density. Understanding T scores and their implications can help healthcare professionals and patients take preventative measures to maintain bone health.

Behçet’s Syndrome: A Multisystem Disorder with Recurrent Oral and Genital Ulcerations

Behçet’s syndrome is a multisystem disorder that primarily affects young people from the Mediterranean region, the Middle East, and the Far East. The syndrome is characterized by recurrent oral and genital ulcerations, as well as ocular involvement. The main pathologic lesion is systemic perivasculitis with early neutrophil infiltration and endothelial swelling. In some patients, vasculitis of the vasa vasorum can result in the formation of pseudo-aneurysms. Anti-Saccharomyces cerevisiae antibodies are present in patients with Behçet’s syndrome. The recurrent aphthous ulcerations are essential for the diagnosis, and a non-specific skin inflammatory reactivity to any scratches or intradermal saline injection (pathergy test) is a common and specific manifestation. Genital ulcers are painful, do not affect the glans penis or urethra, and produce scrotal scars. Thromboembolic events are a component of the disease itself, with superficial or deep peripheral vein thrombosis seen in 30% of patients. Pulmonary emboli are a rare but possible complication.

Other possible diagnoses, such as extra intestinal Crohn’s disease, occult malignancy, systemic lupus erythematosus (SLE), and rheumatoid arthritis, can be ruled out based on the absence of certain clinical features and the presence of anti-Saccharomyces cerevisiae antibodies.

Differential Diagnosis for a Patient with Shoulder and Pelvic Girdle Pain

Polymyalgia rheumatica (PMR) is a likely diagnosis for a patient presenting with shoulder and pelvic girdle pain without muscle weakness and a markedly raised ESR. Rapid improvement of symptoms with corticosteroids and a subsequent fall in ESR confirms the diagnosis. Temporal arthritis, a vasculitis associated with PMR, should also be considered in patients over 50 presenting with headache, vision loss, and jaw claudication. Myositis and dermatomyositis are less likely diagnoses due to the patient’s normal CK and lack of muscle weakness. Rheumatoid arthritis is unlikely given the patient’s age and the classic joint involvement pattern.

Types of Arthritis and Infections that can Cause Tenosynovitis

Tenosynovitis is a condition where the tendon sheath becomes inflamed, causing pain and swelling. It can be caused by various types of arthritis and infections. Here are some of the most common causes:

Systemic Sclerosis: This autoimmune disease causes fibrosis of connective tissue, resulting in hard and thickened skin, swollen digits, and Raynaud’s phenomenon. Tenosynovitis in systemic sclerosis is non-tender and without swelling of the tendons.

Rheumatoid Arthritis: Tenosynovitis due to rheumatoid arthritis causes pain and swelling of tendons. It usually involves the interphalangeal, metacarpophalangeal, and wrist joints, and can cause deformities such as swan neck and Boutonnière’s deformity.

Gout: Gout can cause tenosynovitis, which is very painful and presents with redness and swollen tendons. It typically affects the metatarsophalangeal joints.

Disseminated Gonococcal Infection: This infection can cause acute migratory tenosynovitis, especially in younger adults. Women may be asymptomatic, while men may present with urethral discharge or dysuria.

Reactive Arthritis: This type of arthritis causes pain and swelling of tendons, commonly affecting the knees or sacrum. It is an acutely inflammatory process and would therefore be swollen and tender.

In conclusion, tenosynovitis can be caused by various types of arthritis and infections, and it is important to identify the underlying cause in order to provide appropriate treatment.

Understanding HLA and Autoimmune Diseases

HLA-B27 is a genetic marker associated with ankylosing spondylitis, an autoimmune disease that primarily affects the spine. This disease is more common in males and typically presents in the 20s and 30s. Other autoimmune manifestations, such as anterior uveitis, can also occur in individuals with HLA-B27. Additionally, young men with this genetic marker may be prone to reactive arthritis after chlamydia or gonorrhoeal urethritis.

Rheumatoid factor, on the other hand, is not associated with HLA-B27 or ankylosing spondylitis. While stiffness that improves with exercise may be seen in rheumatoid arthritis, this disease typically affects peripheral joints and does not lead to calcification of the anterior spinal ligament.

Other HLA markers are associated with different autoimmune diseases. HLA-DR3 is linked to type 1 diabetes mellitus, HLA-DR5 is associated with pernicious anaemia and Hashimoto’s thyroiditis, and HLA-B8 is linked to Graves’ disease. Understanding these genetic markers can aid in the diagnosis and management of autoimmune diseases.

Differentiating between Elevated Serum Markers in a Patient with Arthropathy and Hilar Lymphadenopathy

The presence of arthropathy and hilar lymphadenopathy in a patient can be indicative of various underlying conditions. In this case, the patient’s elevated serum markers can help differentiate between potential diagnoses.

Elevated serum angiotensin-converting enzyme (ACE) is a common finding in sarcoidosis, which is likely the cause of the patient’s symptoms. Bilateral hilar lymphadenopathy with or without pulmonary fibrosis is the most typical radiological sign of sarcoidosis. Additionally, acute arthropathy in sarcoidosis patients, known as Löfgren syndrome, is associated with erythema nodosum and fever.

On the other hand, elevated cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) is present in granulomatosis with polyangiitis (GPA), which presents with necrotising granulomatous lesions in the upper and lower respiratory tract and renal glomeruli. It is not typically associated with hilar lymphadenopathy.

Hyperuricaemia and elevated double-stranded (ds) DNA antibody are not relevant to this case, as they are not associated with the patient’s symptoms. Hyperglycaemia is also not a factor in this case.

In conclusion, the combination of arthropathy and hilar lymphadenopathy can be indicative of various underlying conditions. Elevated serum markers can help differentiate between potential diagnoses, such as sarcoidosis and GPA.

Common Bacterial Causes of Septic Arthritis

Septic arthritis can be caused by a variety of bacterial organisms. Among them, Staphylococcus aureus is the most common aetiological agent in Europe and the United States. Streptococcus pyogenes is the next most commonly isolated bacteria, often associated with autoimmune diseases, chronic skin infections, and trauma. Gram-negative bacilli, such as Escherichia coli, account for approximately 10-20% of cases, with a higher prevalence in patients with a history of intravenous drug abuse, extremes of age, or immunocompromised status. Historically, Haemophilus influenzae, S. aureus, and group A streptococci were the most common causes of infectious arthritis in children younger than 2 years, but the overall incidence of H. influenzae is decreasing due to vaccination. Pseudomonas aeruginosa, a less common cause, may affect children, the elderly, intravenous drug users, and immunocompromised patients.

The first-line treatment for ankylosing spondylitis is non-steroidal anti-inflammatories (NSAIDs) like ibuprofen, which should be used alongside physiotherapy and exercise. Long-term NSAID use requires gastro protection with a proton-pump inhibitor. If morning or night pain persists despite NSAIDs, a long-acting preparation can be tried. Tumour necrosis factor (TNF)-alpha inhibitors like etanercept are recommended for poorly controlled ankylosing spondylitis after NSAIDs. Methotrexate is only useful in cases with extensive peripheral joint involvement, which is not mentioned in the vignette. Oral steroids like prednisolone are not first-line management, but intra-articular corticosteroids may be considered for poorly controlled sacroiliitis. Sulfasalazine, a disease-modifying agent used in rheumatoid arthritis, is only useful in ankylosing spondylitis patients with peripheral joint involvement, which is not mentioned in the vignette.