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Granulomatosis with polyangiitis

Synonym: Klinger's syndrome; Wegener's granulomatosis What is granulomatosis with polyangiitis? Granulomatosis with polyangiitis (GPA) - formerly known as Wegener's granulomatosis - is a rare form of vasculitis. Granulomatosis with polyangiitis It is thought to be an autoimmune inflammatory process affecting endothelial cells. It is a multisystem disease which can affect many parts of the body, categorised by the ELK classification: it most commonly presents with lesions in the upper respiratory tract (E indicating ears/nose/throat, almost 100%), lungs (L most patients) and kidneys (K >75%). Many other areas of the body may also be affected, with joint inflammation occurring in 25-50% of all cases. The sinuses, eyes and skin may also be affected. Granulomatosis with polyangiitis epidemiology A study using information from the UK General Practice Research Database reported an overall annual incidence of 8.4/million. One study looking at GPA as a cause of renal vasculitis showed that the annual incidence of such cases in the UK was 5.8/million. The incidence was found to be lower in Japan. The male:female ratio is approximately1.2:1. The condition can occur at any age but peaks between the ages of 35-55. One American study found that the incidence in children was increasing. Another study found that first-degree relatives had a moderately increased risk of developing any autoimmune/inflammatory disease, including specific associations with, for example, multiple sclerosis, Sjögren's syndrome and seropositive rheumatoid arthritis. Granulomatosis with polyangiitis risk factors The higher incidence in winter suggests an infective aetiology but the data are inconclusive. GPA has been linked to parvovirus and to chronic nasal carriage of Staphylococcus aureus. The involvement of the upper airways in this condition has led to the search for possible inhaled allergens, although none has yet been positively identified. Granulomatosis with polyangiitis symptoms As a multisystem disease, GPA often presents with nonspecific symptoms and can be difficult to recognise in primary care. The delay from onset to diagnosis ranges from 2-20 months. GPA symptoms Symptoms may include: Fatigue, malaise. Fever, night sweats. Weakness. Loss of appetite. Weight loss. Rhinorrhoea. Sinusitis. Facial pain. Hoarseness. Cough. Dyspnoea. Wheezing. Chest pain. Joint pains. Hearing loss. Abdominal pain, nausea and vomiting, GI bleeding. In children the renal and respiratory systems are most commonly affected. Signs of GPA The signs found in granulomatosis with polyangiitis occur as a result of the inflammation of the small vessels and may affect any part of the body. The most commonly seen signs relate to the upper and lower airways and the renal tract and may include: Ulcers, sores and crusting, in and around the nose, with destruction of nasal cartilage. Rhinorrhoea, often bloody. Haemoptysis. Haematuria. Subglottic stenosis (38% in one study) - causing hoarseness, stridor, dyspnoea, or cough. Rashes (up to 50%) - often small red/purple raised areas or blister-like lesions, ulcers or nodules. Conjunctivitis, scleritis and episcleritis (32%). Chronic ear infections. Mononeuritis multiplex. Pericarditis. Peritonitis. Unlike polyarteritis nodosa, hypertension and eosinophilia are unusual. Differential diagnosis Granulomatosis with polyangiitis is capable of mimicking numerous other diseases and it is the presence of two or more of the above signs and/or symptoms which must signal the possibility of the diagnosis. Common conditions which enter the differential diagnosis include: Antiglomerular basement membrane (anti-GBM) antibody disease - a rare autoimmune condition in which antibodies are directed towards the glomerular basal membrane and alveoli. Legionella infection. Mixed connective tissue disease. Systemic lupus erythematosus. Other antineutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitides (ie microscopic polyangiitis, Eosinophilic granulomatosis with polyangiitis). Rheumatoid arthritis with systemic vasculitis. Mixed cryoglobulinaemia. Renal vein thrombosis with pulmonary embolism. Investigations FBC, ESR. Serum U&Es. Blood test for cytoplasmic-ANCA (c-ANCA) with autoantibodies directed against proteinase 3 antibodies is seen in 80%-90% of the cases, and the remaining are perinuclear-ANCA (p-ANCA) directed against myeloperoxidase antibodies. Urinalysis for protein, blood and casts. Nasal endoscopy. Lung function tests and flow volume loop looking for subglottic stenosis. CXR looking for cavity formation and pulmonary infiltrates. Chest CT imaging to exclude lung parenchymal involvement. Sinus CT scan to exclude sinus disease. Biopsy of affected tissue, which may include nasal mucosa, lung biopsy, renal biopsy, looking for presence of vasculitis and granulomas. Granulomatosis with polyangiitis treatment and management Medical care Principles of care include rapid diagnosis, early induction of remission, maintenance of remission and prevention of drug toxicity. Investigations to exclude potentially life-threatening and/or vital organ damage should be instituted as a priority. Patients who are asymptomatic and have no organ damage may not need immunosuppressive treatment. Such patients should initially be offered methotrexate to induce remission (with mycophenolate mofetil as an alternative for those intolerant of methotrexate). Cyclophosphamide should be offered to promote remission in patients who have life-threatening and/or vital organ damage. Due to its serious adverse effects (for example, renal, haematological and neurological toxicity), it is normally given as pulsed treatment intravenously every 2-4 weeks. Long-term toxicity is dependent on lifetime cumulative dose which should be ≤25 g. Rituximab is recommended by the National Institute for Health and Care Excellence (NICE) if: Further cyclophosphamide treatment would exceed the maximum cumulative cyclophosphamide dose; or Cyclophosphamide is contra-indicated or not tolerated; or The person has not completed their family, and treatment with cyclophosphamide may materially affect their fertility; or The disease has remained active or progressed despite a course of cyclophosphamide lasting 3-6 months; or The person has had uroepithelial malignancy. Prednisolone is given in addition to cyclophosphamide or rituximab, as it helps to increase patient survival and suppress local disease. Aggressive immunosuppressive therapy is required to control pulmonary and renal involvement. Plasma exchange is sometimes used as an adjunct in these circumstances. Once the patient is in remission, cyclophosphamide should be replaced by azathioprine or methotrexate. Leflunomide or mycophenolate may be given as alternatives if there is intolerance or lack of efficacy. Once the patient has been in remission for at least a year, on maintenance therapy, prednisolone can be tapered off. If the patient remains in remission six months after this, immunosuppressive treatment can be withdrawn. Relapses should be treated by increasing prednisolone and optimising immunosuppressive therapy. Plasma exchange may be needed. Triggers for relapse (for example, infection, malignancy, change in drug therapy) should be considered. The treatment of refractory cases remains a challenge. Rituximab is more effective than cyclophosphamide in these circumstances. Triggers should be considered and the diagnosis reviewed. In 2022 NICE recommended avacopan with a cyclophosphamide or rituximab as an option for treating severe active granulomatosis with polyangiitis in adults. Management with avacopan offers an effective and corticosteroid-free alternative to standard treatment. Surgical care Surgical treatment may be needed for: Nasal deformity. Subglottic stenosis. Obstruction of lacrimal ducts. Bronchial stenoses. Eustachian dysfunction (insertion of grommets). Acute kidney injury (renal transplant). Complications Acute kidney injury. Respiratory failure. Chronic conjunctivitis. Nasal septum perforation. Granulomatosis with polyangiitis prognosis GPA is associated with significant morbidity and mortality either due to irreversible organ dysfunction or due to the consequences of intensive/prolonged use of glucocorticoids and immunosuppressive agents. The average life expectancy for a patient with GPA without any treatment is 5 months, with a 1-year survival rate of less than 20%. Recently advances in treatment have improved the prognosis of patients with GPA, and more effective and less toxic treatment regimes have allowed them to lead a relatively normal life. The prognosis looks more optimistic - 80% of patients now achieve remission although relapses remain frequent (50% at eight years). There are refractory cases remaining resistant to treatment. A poor prognosis is thought to be related to deteriorating renal function and a situation in which the disease process is dominated by systemic vasculitis rather than granulomatosis.

20 Jun 2025

Wound management and suturing

Principles of wound management Assessment. Haemostasis. Analgesia. Skin preparation and wound toilet. Closure. Dressing. Infection prevention. Follow-up. Assessment of wound Mode of injury: blunt, penetrating, blast. Time of injury. Type of wound: puncture, laceration, incision, crush, burst, bite. (Consider removing rings from injured fingers before oedema starts.) Location: proximity to major vessels (potential damage to blood supply for healing), nerves and organs. Shape: linear, curved, stellate, Y-shaped, inverted V, etc. Depth and direction: risk to underlying tissues, skin tension lines. Potential foreign body: suggestive history - whether it will be radio-opaque or require ultrasound scan location. Potential underlying structural injury: bone fracture, tendon rupture, organ perforation. Haemostasis This may be spontaneous. However, it may require: Pressure. Elevation. Tourniquet. Clamp/suture (for arterial bleeders). Analgesia Do not forget analgesia; this is not only humane but facilitates the remainder of the wound management. Local anaesthesia Topical: tetracaine-lidocaine combinations can be used to good effect on wounds in children, even if just to allow infiltration of local anaesthetic. Infiltrative: most often lidocaine (up to 3 mg/kg. NB: a 1% solution contains 10 mg/mL). Caution is generally advised in the use of adrenaline (epinephrine), especially around end arterioles such as those in digits, the penis, etc. However, there is insufficient evidence to justify this fear. If used, the lidocaine dose can be increased up to 7 mg/kg. Skin preparation and wound toilet Disinfect the skin around the wound with antiseptic, but do not put antiseptic inside the wound. Also consider debridement of ragged, non-viable skin edges. If necessary you can trim hair; however, avoid shaving. Simple ointment can be used to flatten any remaining hair away from the wound. Remove foreign bodies but make sure personnel and equipment to control any increase in bleeding are at hand. Irrigation is more important where there is high risk of infection. The aim is to remove foreign matter and bacteria. Normal saline, drinking-quality water, or cooled boiled water can be used. For lacerations that are not visibly contaminated, low-pressure irrigation using a syringe is sufficient. If high pressure is required, use 50-100 mL/cm liquid under pressure from a syringe with a 25G needle. Wound closure Timing Primary closure: immediate closure for simple wounds <12 hours old (24 hours on the face), with opposable edges. Delayed primary closure: if there is high risk of infection, give prophylactic antibiotics and close after approximately four days if there is no infection. Secondary closure: allow the wound to close by itself if a bite (except on the face) or it has separated edges or infection. This may result in increased scarring. Options Take account of the location and severity of the wound and the age, comorbidities and preferences of the wounded person. Suturing (with local anaesthetic) is preferred for wounds longer than 5 cm, or those 5 cm or shorter when: There is likely to be excessive flexing of the wound and tension (for example, over joints or thick dermis), or wetting. Deep dermal sutures are required, to allow low-tension apposition of the wound edges. Tissue adhesives or adhesive strips may be used to close wounds 5 cm or shorter where the risk to infection is low and the wound edges are easily apposed without leaving any dead space, and the wound is not subject to excessive flexing, tension, or wetting: Tissue adhesives are not suitable if any risk factors for infection are present. Always use adhesive strips on pretibial flaps (not tissue adhesives or sutures). Important information Technique tips Generally use interrupted sutures; mattress sutures may be required for larger wounds. First oppose midpoint if linear, or corners if jagged wound. There are special tricks for when there has been skin loss or complex-shaped lacerations. Ensure good bite of tissue taken with needle entering and leaving vertically.. Space sutures about 2-5 mm apart. Suggested sizes and durations Child's face: 6'0 monofilament nylon; remove after 3-5 days. Other parts of children: 5'0 catgut; deep part absorbs and the top part sloughs off after 10-14 days. Adult's face: 5'0 monofilament nylon; remove after five days. Adult hand: 4'0 nylon; remove after seven days. Adult scalp: 4'0 nylon/silk; remove after 3-5 days. Adult arm/trunk/abdomen: 3'0 nylon/silk; remove after 7-10 days. Adult leg: 3'0 nylon; remove after 7-10 days (10-14 days if over a joint). Risk factors for delayed healing Size, location and motion of wound. Age. Genetics. Ethnicity. Marfan's syndrome, connective tissue disorders. Nutrition; deficiencies in protein, vitamins A, C, E, B1 (thiamine), other B vitamins, and zinc have been shown to retard healing. However, supplements to non-deficient patients probably have little or no benefit. Local infection. Ischaemia. Glucocorticoid therapy. Diabetes mellitus. Smoking. Foreign bodies. Wound dressings The first layer in contact with the wound surface should be non-adherent - for example, a lubricated gauze with interstices. Occlusive dressings can lead to maceration with retained fluid. If there is a large amount of exudate, the next layer should be absorbent material such as alginate or foam. Finally, soft gauze rolls tape can be used to secure the initial materials in place. Dressings may not be necessary if the wound is dry and extra protection is not required. Wound infection Signs and symptoms Increasing local inflammation - rubor, dolor, calor and tumour. Discharge/collection of pus. Systemic signs such as fever or malaise. Risk factors Delayed presentation (>12 hours). Foreign bodies. Heavily soiled wounds. Bites (especially human, cats). Puncture wounds (especially on the foot). Intra-oral wounds. Open fractures/exposed tendons. Crush wounds. Antibiotic usage in wound management Antibiotics should be used if there are already signs of infection, or if there is a high-risk of infection, such as those where there are comorbidities, gross contamination, involvement of deeper structures, stellate wounds, and selected bite wounds. Take a swab of the wound before starting antibiotic treatment. If the wound is contaminated with high-risk material (for example, soil, faeces, saliva, or purulent exudates), treat with co-amoxiclav. If the person is allergic to penicillin, treat with erythromycin or clarithromycin combined with metronidazole. If the wound is clean, treat with flucloxacillin. If the person is allergic to penicillin, treat with erythromycin or clarithromycin. Mode of delivery is usually oral, unless there are systemic signs or rapid spread. Topical antibiotic ointment is an option. Be aware of side-effects/resistance. Follow-up Give written advice on wound care. Check for healing progress and signs of infection at 48-96 hours. Removal of sutures, if present, at the appropriate time.

19 Jun 2025

Peripheral oedema

What is peripheral oedema? Oedema is an accumulation of interstitial fluid. The volume of fluid in the interstitial space is normally kept constant at around 20% of body weight. Several factors control the direction of flow of interstitial fluid including hydrostatic pressure, oncotic pressure, endothelial integrity, and lymphatic systems. These components are described by Starling’s law where fluid movement across capillaries is proportional to capillary permeability, trans-capillary hydrostatic pressure differences, and trans-capillary oncotic pressure differences. Capillary pressure forces fluid from the capillaries into the interstitium where the arterial end pressure is higher than the venous end. The interstitial fluid pressure varies based on the density of tissues and it can be a positive or negative value, with positive values being due to fluid forced into the capillary and negative values being fluid forced into the interstitium. Plasma oncotic pressure is due to proteins that cannot easily pass between the interstitium and plasma, and therefore exert an osmotic effect across capillary walls. Albumin is the most significant plasma protein. A small amount of protein is found in the interstitium and forces some fluid out of capillary walls. This force is the interstitial oncotic pressure. The balance of these factors contributes to the formation of oedema. Peripheral oedema types Oedema is referred to as 'pitting' when (after pressing on the affected skin), an indentation remains after the source of pressure has been removed. (For example, if you press gently on oedematous skin with your finger, then stop doing so, you can still see and feel the finger-shaped dent left behind.) This is the classic type of oedema caused by fluid accumulation. Non-pitting oedema occurs in conditions such as lymphoedema, myxoedema and lipoedema. Peripheral oedema causes Pitting-dependent oedema This is oedema of the ankle if mobile, sacral when bed-bound. Immobility: Increased fluid pressure from venous stasis. Varicose veins. Obesity: Increased fluid pressure from sodium and water retention; should not to be confused with non-pitting lymphoedema. Cardiac: Increased fluid pressure: right heart failure, constrictive pericarditis. Drugs: Increased fluid pressure from sodium and water retention: calcium antagonists, non-steroidal anti-inflammatory drugs (NSAIDs), prolonged steroid therapy, insulin. Hepatic: Decreased oncotic pressure due to hypoalbuminaemia. Also increased capillary permeability from systemic venous hypertension. Renal: Decreased oncotic pressure from protein loss, and increased fluid pressure from sodium and water retention: acute nephritic syndrome, nephrotic syndrome. Gastrointestinal: Decreased oncotic pressure: starvation, malnutrition, malabsorption, protein-losing enteropathy (for example, Crohn's disease, ulcerative colitis, tumours of stomach and colon, coeliac disease and other intestinal allergies). Obstructive sleep apnoea: Increased capillary hydrostatic pressure due to pulmonary hypertension. Pregnancy: Increased fluid pressure both from sodium and water retention and venous stasis from pelvic obstruction. High-altitude illness: Oedema of face, hands and ankles may occur. Cerebral and pulmonary oedema, however, are usually of greater concern if disease progresses. Idiopathic oedema: Associated with cyclical high lymph volume overload, or dynamic insufficiency: usually in a woman aged 20-40 years. Variable and not related to menstrual periods. Diagnosis is based on the exclusion of other causes of oedema. Post-thrombotic syndrome: Late complication of deep vein thrombosis (DVT) which occurs in up to two thirds of patients. May present with pain, oedema, hyperpigmentation, and even skin ulceration. May result from remaining venous obstructions, from reflux, or both. Rate of reflux is highest during the 6-12 months after an acute DVT. It may be temporary and self-limiting or not resolve, and persist at variable severity. Pitting localised limb oedema Deep vein thrombosis (DVT). Compression of large veins by tumour or lymph nodes. Following hip replacement or knee replacement. Chronic venous insufficiency. May be unilateral or bilateral. Usually unilateral predominance. Local infection, trauma (including burns, which may also cause generalised oedema because of protein loss), animal bites or stings. Non-pitting lower-limb oedema Hypothyroidism (mucopolysaccharide deposition). Lymphoedema: Blocked lymph channels: surgical damage, radiation, malignant infiltration, infectious (for example, filariasis), congenital (for example, Milroy's disease). Lipoedema. Allergy: Increased capillary permeability: angio-oedema. Peripheral oedema symptoms Assessment should include: Duration: swelling due to venous insufficiency is usually a long-standing problem. Distribution of oedema: Dependent oedema in an otherwise well patient suggests a benign cause such as immobility or varicose veins. Pulmonary and ankle oedema are typical of cardiac failure. Hands and face, which is most marked after lying down, occurs in hypoproteinaemia. Ascites in liver failure, nephrotic syndrome, protein malnutrition. Unilateral swelling, particularly of the calf, suggests a DVT. Oedema in angio-oedema is mainly restricted to the face and lips, although any part of the body may be affected. Hydroceles: fluid often accumulates in the scrotal sac - for example, in nephrotic syndrome. Pitting-dependent oedema will become sacral if bed-bound. Associated symptoms: breathlessness of recent onset may be due to cardiac failure, anaemia, lung cancer or pleural effusions (for example, from nephrotic syndrome). Past history: coronary heart disease, chronic lung disease, DVT (past history could lead to venous insufficiency). Medication. Examination is directed towards assessment of the cause of oedema and therefore a full assessment including the cardiovascular system and abdomen is required. Unilateral ankle oedema should raise suspicion of a DVT but oedema may be bilateral in inferior vein obstruction and, in cases of bilateral oedema, one side may be more affected and therefore more obvious than the other. By James Heilman, MD (own work) via Wikimedia Commons Investigations A thorough history and examination along with a urine dipstick test will usually be sufficient to establish the cause, but the following may be required if clinical findings suggest: Urine testing: (a combination of profuse proteinuria and oedema, with hypoalbuminaemia confirmed on blood testing, is pathognomonic of nephrotic syndrome). Haemoglobin (anaemia may be a cause or aggravating factor of heart failure). Renal function and electrolytes (renal disease such as acute kidney injury, chronic kidney disease, nephrotic syndrome, nephritic syndrome, etc). LFTs (liver failure; may show hypoproteinaemia in cirrhosis, nephrotic syndrome, protein-losing enteropathy). TFTs (for hypothyroidism). Abdominal/pelvic ultrasound: will reveal, for example, pelvic tumour, ascites, liver metastases. CXR: if heart failure or lung malignancy is suspected. ECG: if heart failure is suspected. D-dimer and duplex ultrasound scan where DVT is suspected. Duplex ultrasonography may also be useful for confirming chronic venous insufficiency. Lymphoscintigraphy has a 92% sensitivity and 100% specificity for identifying lymphoedema and is considered the gold standard for diagnosis. Magnetic resonance imaging (MRI) may be indicated if ultrasonography is not conclusive and suspicion of DVT is high, or as an alternative to lymphoscintigraphy in evaluation of lymphoedema. It may also be required if tumours are found. Algorithm The following algorithm may be helpful in assessing peripheral oedema. Peripheral oedema treatment Treatment is based on the cause. Empirical treatment with diuretics is inappropriate in the absence of a clear diagnosis.

19 Jun 2025

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