Nursing(Degree) · First Year · NUR101
Anatomy and Physiology
Anatomy and Physiology is one of the most important foundation courses for nursing students in Zambia. This course helps you understand how the human body is structured and how it functions, from major organs to body systems such as the nervous, cardiovascular, respiratory, and digestive …
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Anatomy and Physiology is one of the most important foundation courses for nursing students in Zambia. This course helps you understand how the human body is structured and how it functions, from major organs to body systems such as the nervous, cardiovascular, respiratory, and digestive systems. A strong grasp of this subject is essential for exams set by nursing schools and regulatory bodies in Zambia. At 25schools, we simplify complex anatomy concepts using exam-focused questions, diagrams, and clear explanations designed for nursing exams. Whether you are preparing for tests, assignments, or final examinations, this course builds the clinical understanding every successful nurse needs. Start studying with 25schools today and gain the confidence to excel in anatomy and physiology exams.
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Psoriatic Arthritis
Psoriatic arthritis (PsA) is chronic inflammatory arthritis associated with psoriasis, part of spondyloarthritis family—heterogeneous disease with five clinical domains: peripheral arthritis, axial disease, enthesitis, dactylitis, skin/nail psoriasis. For Zambian students, relevant for dermatology-rheumatology interface; often unrecognized. Pathophysiology: IL-23/IL-17 axis, TNF, genetic (HLA-B27 axial, HLA-Cw0602 psoriasis). Enthesitis central. Clinical: PSORIASIS precedes arthritis in 70% (by years), concurrent 15%, arthritis precedes 15%. SKIN: plaque psoriasis (90%), scalp, extensor surfaces, umbilicus, intergluteal. NAIL: pitting, onycholysis, hyperkeratosis, oil-drop discoloration—strongly associated with PsA (80% PsA vs 30% psoriasis alone). PERIPHERAL ARTHRITIS (95%): five patterns—ASYMMETRIC OLIGOARTHRITIS (most common, 50-70%, knee, ankle, MCP, PIP), SYMMETRIC POLYARTHRITIS (25%, RA mimic—distinguish: DIP involved, RF negative, nail changes), DIP PREDOMINANT (5%, highly specific), ARTHRITIS MUTILANS (5%, severe osteolysis, telescoping digits, pencil-in-cup deformity), AXIAL (25-50%, sacroiliitis, spondylitis—less severe than AS). ENTHESITIS (30-50%): Achilles, plantar fascia, patellar tendon, iliac crest. DACTYLITIS (30-50%): sausage digit—diffuse swelling entire digit (flexor tenosynovitis), highly characteristic. EXTRA-ARTICULAR: uveitis (bilateral, insidious, less painful than AS), IBD. Diagnosis: CLINICAL—inflammatory arthritis + psoriasis (personal/family history) + characteristic features (DIP, dactylitis, enthesitis, RF negative, radiographic changes). Radiographs: proliferative bone changes (distinguishing from RA)—periostitis, ankylosis, pencil-in-cup deformity, osteolysis, sacroiliitis (asymmetric). MRI/ultrasound: enthesitis, synovitis, dactylitis. CASPAR criteria: inflammatory articular disease + ≥3 points—current psoriasis (2), personal/family history psoriasis (1), nail dystrophy (1), RF negative (1), dactylitis (1), radiographic juxta-articular new bone formation (1). Management: MULTIDOMAIN APPROACH. MILD PERIPHERAL: NSAIDs, intra-articular corticosteroids. MODERATE-SEVERE PERIPHERAL: csDMARDs—methotrexate (skin + joints), leflunomide, sulfasalazine. AXIAL/ENTHESITIS/DACTYLITIS: csDMARDs INEFFECTIVE—TNF inhibitors first-line (adalimumab, etanercept, infliximab, golimumab, certolizumab). IL-17 inhibitors (secukinumab, ixekizumab), IL-12/23 inhibitors (ustekinumab), JAK inhibitors (tofacitinib) alternatives. In Zambia: csDMARDs available, biologics largely inaccessible. Prognosis: chronic, progressive without treatment; modern therapy achieves remission/minimal disease activity in 40-60%. Zambian context: underdiagnosed; consider PsA in patients with psoriasis and inflammatory arthritis; methotrexate accessible for peripheral disease; axial/entheseal disease challenging without biologics.
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Reactive Arthritis
Reactive arthritis (ReA) is spondyloarthritis arising 1-4 weeks after certain gastrointestinal or genitourinary infections, classically presenting with arthritis, urethritis/cervicitis, conjunctivitis—'incomplete Reiter's' (only 1/3 have classic triad). For Zambian students, important differential diagnosis in sexually active adults and post-dysentery. Pathophysiology: preceding infection (GI: Shigella, Salmonella, Campylobacter, Yersinia; GU: Chlamydia trachomatis) in genetically susceptible (HLA-B27 30-50%). Viable organisms NOT in joint—immune-mediated response to bacterial antigens cross-reacting with self. Clinical: ONSET 1-4 weeks post-infection (may be subclinical). ARTHRITIS: asymmetric oligoarthritis (80-90%), lower extremity predominant (knee, ankle, foot), acute, inflammatory. Dactylitis (sausage toe). Enthesitis (Achilles, plantar fascia). Axial: sacroiliitis (unilateral/bilateral), spondylitis (non-marginal syndesmophytes, asymmetric). MUCOCUTANEOUS: urethritis/cervicitis (sterile, dysuria, discharge), conjunctivitis (mild, self-limited), circinate balanitis (painless, shallow ulcers on glans penis), keratoderma blennorrhagicum (pustular/plaques palms, soles—clinically identical to pustular psoriasis), oral ulcers (painless). OTHER: nail changes (pitting, onycholysis), cardiac (conduction abnormalities, aortitis), renal (IgA nephropathy). Diagnosis: CLINICAL—acute asymmetric oligoarthritis + recent infection + characteristic extra-articular features. No diagnostic test. HLA-B27 supports, not diagnostic. Infection workup: stool culture, Chlamydia NAAT (urine, urethral, cervical), serology (Yersinia, Salmonella, Campylobacter). Radiographs: sacroiliitis, periostitis, large asymmetric syndesmophytes. Management: TREAT PRECIPITATING INFECTION if active (azithromycin for Chlamydia). ARTHRITIS: NSAIDs first-line. Intra-articular corticosteroids. csDMARDs (sulfasalazine, methotrexate) for persistent peripheral arthritis (>3-6 months). TNF inhibitors for refractory, severe, axial disease. MUCOCUTANEOUS: topical corticosteroids, keratoderma—topical vitamin D analogues, retinoids. Prognosis: 50% remission <6 months, 30% prolonged intermittent, 20% chronic severe. HLA-B27 associated with chronicity, axial disease, worse outcome. Zambian context: high prevalence of enteric infections, STIs; consider ReA in young adult with acute oligoarthritis; diagnose clinically; NSAIDs, sulfasalazine accessible.
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Enteropathic Arthritis
Enteropathic arthritis (EA) is spondyloarthritis associated with inflammatory bowel disease (IBD: Crohn's disease, ulcerative colitis) or other GI conditions. For Zambian students, important consideration in patients with IBD or unexplained chronic diarrhoea. Pathophysiology: shared genetic susceptibility (HLA-B27 axial, NOD2/CARD15 Crohn's), gut inflammation → increased intestinal permeability → bacterial antigen translocation → immune activation → synovial inflammation. Clinical: TWO PATTERNS—PERIPHERAL (60-70%): acute, self-limited, asymmetric oligoarthritis, large joints (knee, ankle, wrist), correlates with IBD activity, non-erosive. AXIAL (10-20%): sacroiliitis, spondylitis, clinically/radiographically INDISTINGUISHABLE from ankylosing spondylitis, DOES NOT correlate with IBD activity, may precede bowel symptoms. MUCOCUTANEOUS: erythema nodosum (Crohn's), pyoderma gangrenosum (UC). Diagnosis: CLINICAL—inflammatory arthritis + confirmed IBD. Radiographs: sacroiliitis, syndesmophytes (asymmetric, non-marginal). Management: PERIPHERAL: treat IBD—arthritis improves with bowel control. NSAIDs with CAUTION (may exacerbate IBD). Intra-articular corticosteroids. Sulfasalazine effective for peripheral arthritis + colitis. Methotrexate. TNF inhibitors (infliximab, adalimumab) for refractory peripheral + treat IBD. AXIAL: NSAIDs (caution), TNF inhibitors (effective for axial + IBD). IL-12/23 inhibitors (ustekinumab) for Crohn's + arthritis. Zambian context: IBD less common but increasing recognition; consider EA in patient with chronic diarrhoea, weight loss, and arthritis; sulfasalazine accessible; TNF inhibitors largely unavailable.
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Rhabdomyolysis
Rhabdomyolysis is life-threatening syndrome of skeletal muscle injury and necrosis with release of intracellular contents into circulation, causing acute kidney injury, electrolyte disturbances, and compartment syndrome. For Zambian students, critical in trauma, prolonged immobilization, heat illness, malaria, HIV, and statin use. Pathophysiology: direct muscle injury (crush, burns, electrocution) OR metabolic demands > ATP supply (seizures, extreme exertion, hyperthermia) OR toxins/medications (statins, antipsychotics, alcohol, snake envenomation) OR infection (influenza, HIV, malaria, pyomyositis) OR genetic (glycogen storage diseases, malignant hyperthermia). Sarcolemmal disruption → release of myoglobin, creatine kinase (CK), aldolase, lactate dehydrogenase, potassium, phosphate, urate into circulation. MYOGLOBIN—direct proximal tubular cytotoxicity, cast formation (Tamm-Horsfall protein + myoglobin), intraluminal obstruction, renal vasoconstriction → ACUTE KIDNEY INJURY (10-50%). Clinical: MUSCLE PAIN, WEAKNESS, TENDERNESS, SWELLING—may be absent in 50%. Dark urine (cola-coloured, myoglobinuria). Systemic: fever, malaise, nausea, vomiting, confusion. Complications: AKI (oliguric, may require dialysis), COMPARTMENT SYNDROME (muscle edema in rigid fascia), hyperkalaemia (life-threatening arrhythmias), hypocalcaemia (calcium deposition in injured muscle), DIC, hepatic dysfunction. Diagnosis: SERUM CK >5x normal (≥1000 U/L), peak 12-72h post-injury—correlates with AKI risk (CK >5000-10,000). URINALYSIS: dipstick blood + with few RBCs (myoglobinuria). Serum/urine myoglobin (rapid clearance, lower sensitivity). Electrolytes: K+, PO4³⁻ elevated, Ca²⁺ early low (later high). Renal: creatinine elevated. Management: AGGRESSIVE IV FLUID RESUSCITATION—cornerstone therapy. Goal: urine output 200-300 mL/h. Isotonic saline (0.9% NaCl), avoid potassium-containing fluids. Monitor urine output, electrolytes, CK q6-12h. ALKALINIZE URINE: sodium bicarbonate to urine pH >6.5 (controversial, may reduce cast formation, correct metabolic acidosis). Avoid nephrotoxins: NSAIDs, ACE inhibitors, aminoglycosides. MANNITOL: no proven benefit, may worsen compartment syndrome. Treat hyperkalaemia emergently. Hypocalcaemia: treat ONLY if symptomatic or severe hyperkalaemia (calcium stabilizes myocardium). Compartment syndrome: EMERGENCY FASCIOTOMY. AKI: haemodialysis indication (refractory hyperkalaemia, acidosis, volume overload, uraemia). Prognosis: excellent with prompt, aggressive fluid resuscitation. Delayed treatment → AKI, mortality 5-10%. Zambian context: high risk—prolonged labour, prolonged surgery, crush injury, malaria, HIV-associated myositis, traditional medicine toxins, snakebite. Prevention: early, aggressive hydration in at-risk patients; avoid NSAIDs in volume-depleted; rhabdomyolysis awareness.
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Muscular Dystrophies
Muscular dystrophies (MD) are inherited, progressive disorders characterized by muscle fibre degeneration, weakness, and replacement by fibrofatty tissue. For Zambian students, essential for paediatric neurology, differential diagnosis of weakness. Pathophysiology: genetic defects in sarcolemmal, extracellular matrix, or nuclear membrane proteins → sarcolemmal instability, impaired membrane repair, increased susceptibility to contraction-induced injury → progressive necrosis, failed regeneration, fibroadipose replacement. Duchenne MD: X-linked recessive, dystrophin gene mutations (out-of-frame, absent dystrophin). Incidence 1/5000 males. Onset <5 years: delayed walking, waddling gait, Gowers' sign (climbing up thighs to stand), calf pseudohypertrophy. Progressive: loss of ambulation by 12, respiratory failure/cardiomyopathy by 20-30. Becker MD: X-linked, in-frame dystrophin mutations (reduced/abnormal dystrophin). Later onset (childhood-adolescence), slower progression, ambulation >16 years, variable severity. Myotonic dystrophy (DM1): autosomal dominant, CTG repeat expansion (DMPK gene). MULTISYSTEM: myotonia (delayed relaxation after contraction, grip/percussion), distal > proximal weakness, facial weakness (hatchet facies), ptosis, dysarthria. Cardiac conduction defects, cataracts, insulin resistance, frontal balding, cognitive impairment. Facioscapulohumeral MD: autosomal dominant, DUX4 gene, facial weakness (cannot close eyes, whistle), scapular winging, humeral weakness, foot drop. Limb-girdle MD: heterogeneous, autosomal recessive/dominant, pelvic/shoulder girdle weakness, preserved facial strength. Diagnosis: SERUM CK: markedly elevated in DMD/BMD (50-100x normal), moderately elevated in LGMD, normal-mild in FSHD. EMG: myopathic units, fibrillations. MUSCLE BIOPSY: fibre size variation, necrosis, regeneration, fibrosis, fatty infiltration. Immunostaining: dystrophin absent/DMD, reduced/abnormal BMD. GENETIC TESTING: definitive. Management: NO CURE. CORTICOSTEROIDS (prednisone, deflazacort) in DMD: prolong ambulation 2-3 years, preserve pulmonary/cardiac function. PHYSICAL THERAPY: stretching (contracture prevention), orthoses (ankle-foot), mobility aids. CARDIAC: ACE inhibitors, beta-blockers for cardiomyopathy; annual echocardiogram. PULMONARY: cough assist, non-invasive ventilation (respiratory failure). SCOLIOSIS SURGERY. GENE THERAPY: emerging (micro-dystrophin, exon skipping). Zambian context: limited genetic testing, specialist neurology, multidisciplinary care. Supportive care: physiotherapy, contracture prevention, respiratory care, cardiac monitoring. Genetic counselling.
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Myasthenia Gravis
Myasthenia gravis (MG) is autoimmune disorder of neuromuscular junction, caused by antibodies against acetylcholine receptors (AChR) or receptor-associated proteins, leading to fatigable weakness. For Zambian students, essential for neurology, critical care, and thymus pathology. Pathophysiology: AChR antibodies (80%) → AChR blockade, cross-linking/internalization, complement-mediated destruction → reduced functional AChRs at postsynaptic membrane. MuSK antibodies (5-10%, female predominant, oculo-bulbar, neck, respiratory). LRP4 antibodies, seronegative. Thymic abnormalities: thymoma (10-15%), thymic hyperplasia (50-70%). Clinical: FATIGABLE WEAKNESS—worse with repetitive use, improves with rest, diurnal variation (worse evening). OCULAR: ptosis (unilateral/bilateral, asymmetric), diplopia—presentation in 50%, isolated ocular MG (15%). BULBAR: dysarthria (nasal speech), dysphagia, fatigable chewing, facial weakness (snarling smile). LIMB: proximal > distal, upper > lower. RESPIRATORY: dyspnoea, orthopnoea—MYASTHENIC CRISIS (respiratory failure requiring ventilation), triggered by infection, surgery, medication, pregnancy, tapering immunotherapy. Diagnosis: CLINICAL—fatigable weakness. ICE PACK TEST: improves ptosis. ACETYLCHOLINESTERASE INHIBITOR TEST: edrophonium (Tensilon) IV—brief improvement (30-60s)—now rarely used, neostigmine IM alternative. SEROLOGY: AChR antibodies (80% generalized, 50% ocular), MuSK antibodies. ELECTROPHYSIOLOGY: repetitive nerve stimulation—decremental response (>10% decline). Single-fibre EMG—increased jitter (most sensitive). THORACIC IMAGING: CT chest—thymoma, thymic hyperplasia. Management: SYMPTOMATIC: pyridostigmine (acetylcholinesterase inhibitor)—first-line, 30-60mg q4-6h. CHRONIC IMMUNOSURPRESSION: corticosteroids (prednisone)—slow titration to avoid exacerbation. Steroid-sparing: azathioprine, mycophenolate, methotrexate, cyclosporine. RAPID IMMUNOMODULATION: plasma exchange, IV immunoglobulin—myasthenic crisis, pre-thymectomy, severe exacerbation. THYMECTOMY: indicated for thymoma, consideration in non-thymomatous AChR+ generalized MG (<60y). MONOCLONAL ANTIBODIES: rituximab (MuSK+, refractory), eculizumab (complement inhibitor, refractory AChR+). MYASTHENIC CRISIS: ICU admission, airway protection, non-invasive/mechanical ventilation, IVIG/PLEX, treat trigger, hold pyridostigmine (secretions). Avoid: aminoglycosides, fluoroquinolones, macrolides, beta-blockers, magnesium, quinine—may worsen weakness. Prognosis: excellent with modern therapy—normal lifespan, good function. Crisis mortality <5%. Zambian context: limited serology, electrophysiology, ICU capacity. Clinical diagnosis—fatigable ptosis, diplopia, bulbar weakness. Pyridostigmine accessible. Prednisone accessible. Azathioprine accessible. Thymectomy available tertiary centres. Crisis management challenging.
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Polymyositis and Dermatomyositis
Idiopathic inflammatory myopathies—polymyositis (PM) and dermatomyositis (DM)—are autoimmune disorders causing progressive, symmetric proximal muscle weakness, with characteristic skin manifestations in DM. For Zambian students, important differential diagnosis for proximal weakness; DM associated with malignancy requires screening. Pathophysiology: DM—humoral-mediated, complement attack on endomysial capillaries → capillary depletion, ischaemia, perifascicular atrophy. PM—cell-mediated, CD8+ cytotoxic T cells invade non-necrotic muscle fibres expressing MHC-I. Interstitial lung disease common (anti-synthetase syndrome). DM: cutaneous small vessel vasculopathy. Clinical: MUSCLE: symmetric proximal weakness—hip girdle (difficulty rising from chair, climbing stairs), shoulder girdle (overhead activities), neck flexors. Distal weakness late. Myalgia in 50%. Dysphagia (pharyngeal, oesophageal skeletal muscle). Respiratory: dyspnoea (aspiration, ILD). Cardiac: conduction defects, myocarditis. SKIN (DM): HELIOTROPE RASH—purple, periorbital oedema. GOTTRON PAPULES—violaceous papules over MCP, PIP, DIP extensor surfaces. GOTTRON SIGN—erythema over extensor surfaces (knees, elbows, malleoli). SHAWL SIGN, V-SIGN (photodistributed poikiloderma). MECHANIC'S HANDS—hyperkeratosis, fissuring lateral/ palmar fingers (anti-synthetase). CALCINOSIS (juvenile DM). ANTISYNTHETASE SYNDROME: anti-Jo-1, ILD, mechanic's hands, Raynaud's, arthritis, fever. MALIGNANCY-ASSOCIATED DM: ovarian, lung, colorectal, pancreatic, gastric, breast, NHL. Highest risk within 2 years before/after diagnosis. Diagnosis: SERUM CK: elevated (PM/DM) — may be normal in 10%. EMG: myopathic units, fibrillations, sharp waves (irritable myopathy). MUSCLE BIOPSY: essential. DM—perifascicular atrophy, perivascular inflammation, MAC deposition. PM—endomyseal CD8+ T cells invading non-necrotic fibres. MYOSITIS-SPECIFIC ANTIBODIES: anti-Jo-1, anti-Mi-2, anti-SRP, anti-TIF1γ, anti-NXP2, anti-MDA5 (clinically amyopathic DM, rapidly progressive ILD). MALIGNANCY SCREENING (DM): age-appropriate, CT chest/abdomen/pelvis, mammography, pelvic ultrasound, colonoscopy. Management: CORTICOSTEROIDS: prednisone 0.5-1mg/kg/day, slow taper over 9-12 months (high relapse risk). STEROID-SPARING: methotrexate, azathioprine, mycophenolate (first-line). IVIg: refractory, severe, or dysphagia (DM). Rituximab: refractory. ILD: aggressive immunosuppression, mycophenolate, cyclophosphamide, tacrolimus. CALCINOSIS: difficult, colchicine, diltiazem, bisphosphonates, surgery. PHYSICAL THERAPY: essential, gentle range of motion, strengthening. Prognosis: 5-year survival 80-90%. Mortality: ILD, malignancy, cardiac, infection. Zambian context: limited muscle biopsy, serology, EMG; clinical diagnosis—proximal weakness, rash (DM), elevated CK. High suspicion for HIV-associated myopathy (similar presentation). Malignancy screening essential for DM.
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Systemic Sclerosis (Scleroderma)
Systemic sclerosis (SSc) is chronic autoimmune connective tissue disease characterized by microvascular injury, immune dysregulation, and progressive fibrosis of skin and internal organs. For Zambian students, important for rheumatology, pulmonary hypertension, and renal crisis recognition. Pathophysiology: vascular (early: endothelial injury, apoptosis, capillary rarefaction, intimal proliferation, Raynaud's), immune (autoantibodies, T cells, macrophages, cytokines—TGF-β, PDGF, IL-6), fibroblast activation → excessive extracellular matrix deposition (collagen, proteoglycans) → progressive fibrosis. Classification: LIMITED CUTANEOUS (lcSSc, 60%): skin thickening DISTAL to elbows/knees, face. CREST syndrome: Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangiectasia. Slow progression, late pulmonary hypertension. DIFFUSE CUTANEOUS (dcSSc, 40%): skin thickening PROXIMAL to elbows/knees, trunk. Rapid progression, early ILD, renal crisis, cardiac. SYSTEMIC SCLEROSIS SINE SCLERODERMA: internal organ fibrosis without skin involvement. Clinical: VASCULAR: RAYNAUD'S PHENOMENON (95%)—vasospasm digits with cold/stress, triphasic colour change (white→blue→red). Digital ulcers, pitting scars, gangrene. Telangiectasia (face, hands, mucous membranes). SKIN: sclerodactyly (tapered fingers, tight skin), hand deformity (fixed flexion contractures), facial (tight skin, beak nose, microstomia, radial furrowing). Calcinosis (fingertips, pressure points). MUSCULOSKELETAL: arthralgia, flexion contractures, tenosynovitis, myopathy. GASTROINTESTINAL (90%): esophageal dysmotility (dysphagia, reflux), gastroparesis, small bowel dysmotility (pseudo-obstruction, bacterial overgrowth), intestinal telangiectasia (bleeding), faecal incontinence. PULMONARY: INTERSTITIAL LUNG DISEASE (dcSSc, anti-Scl-70, 30-50%)—NSIP pattern, progressive fibrosis, leading cause of death. PULMONARY ARTERIAL HYPERTENSION (lcSSc, anti-centromere, 15-30%)—leading cause death. CARDIAC: myocardial fibrosis, diastolic dysfunction, conduction defects, pericardial effusion. RENAL: SCLERODERMA RENAL CRISIS (dcSSc, rapid progressive skin, anti-RNA polymerase III, 10-15%)—malignant hypertension, acute kidney injury, microangiopathic haemolytic anaemia, hyperreninaemia. PREVENTS: prompt ACE inhibitor (captopril) regardless creatinine. Diagnosis: CLINICAL—skin thickening proximal to MCPs. AUTOANTIBODIES: anti-centromere (lcSSc, PAH), anti-Scl-70 (topoisomerase I, dcSSc, ILD), anti-RNA polymerase III (dcSSc, renal crisis, malignancy). NAILFOLD CAPILLAROSCOPY: dilated loops, dropout, haemorrhages. PFTs: FVC (ILD), DLCO (PAH, out of proportion to FVC). ECHO: PAH screening. HRCT: ILD (NSIP, UIP). BARIUM SWALLOW: dilated oesophagus, dysmotility. Management: DISEASE-MODIFYING: methotrexate (early dcSSc skin), mycophenolate (ILD), cyclophosphamide (ILD), tocilizumab (ILD, skin), nintedanib (ILD, antifibrotic). No proven therapy for fibrosis reversal. VASCULAR: calcium channel blockers (Raynaud's), PDE5 inhibitors (sildenafil, tadalafil—Raynaud's, PAH), prostacyclin analogues (IV epoprostenol, PAH; IV iloprost—Raynaud's, ulcers), endothelin receptor antagonists (bosentan, ambrisentan—PAH, ulcers). GI: PPI (high-dose), prokinetics, rotating antibiotics (bacterial overgrowth). RENAL CRISIS: ACE inhibitor (captopril) EMERGENCY—continue despite rising creatinine (dialysis may be temporary). Rituximab: refractory. AUTOLOGOUS STEM CELL TRANSPLANT: severe, progressive dcSSc. Prognosis: lcSSc: normal lifespan (PAH risk). dcSSc: 10-year survival 60-70%; poor prognosis: male, older onset, diffuse skin, ILD, PAH, renal crisis, cardiac. Zambian context: limited serology, nailfold capillaroscopy, PAH therapies, ILD expertise. Emphasis: clinical recognition—Raynaud's + puffy fingers + GERD + skin thickening. Screen for ILD (symptoms, PFTs), PAH (echo). Renal crisis: malignant hypertension + AKI + MAHA = scleroderma renal crisis until proven otherwise—ACE inhibitor IMMEDIATELY. Manage GI reflux, dysmotility symptomatically.
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Sjögren's Syndrome
Sjögren's syndrome (SS) is chronic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands, causing keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth). May be primary or secondary (associated with RA, SLE, SSc). For Zambian students, important for differential diagnosis of dry eyes/mouth, fatigue, arthralgia. Pathophysiology: autoimmune epithelitis—CD4+ T cell, B cell infiltration of lacrimal, salivary glands → glandular destruction, dysfunction. B cell hyperreactivity: autoantibodies (anti-Ro/SSA, anti-La/SSB), hypergammaglobulinaemia, increased lymphoma risk (5-10%, highest among autoimmune diseases). Clinical: OCULAR: keratoconjunctivitis sicca—dryness, grittiness, burning, foreign body sensation, photosensitivity, blurred vision. ORAL: xerostomia—dry mouth, dysphagia, dysgeusia, increased caries, oral candidiasis, parotid gland enlargement (bilateral, firm, nontender). OTHER EXOCRINE: nasal dryness, dry skin, vaginal dryness. MUSCULOSKELETAL: arthralgia, non-erosive arthritis (50-70%). FATIGUE (70-80%). SYSTEMIC (30-40%): Raynaud's, cutaneous vasculitis (palpable purpura), interstitial lung disease (LIP, NSIP), renal (interstitial nephritis, RTA type I), neurologic (peripheral neuropathy, mononeuritis multiplex, cranial neuropathy, transverse myelitis), haematologic (anaemia, leukopenia, thrombocytopenia, cryoglobulinaemia). LYMPHOMA: MALT lymphoma (parotid), diffuse large B-cell—persistent parotid enlargement, lymphadenopathy, splenomegaly, cryoglobulinaemia, vasculitis. Diagnosis: CLINICAL—sicca symptoms + objective ocular/oral dryness + autoantibodies + labial salivary gland biopsy focus score ≥1 (positive). AUTOANTIBODIES: anti-Ro/SSA (50-70%), anti-La/SSB (30-50%)—specific for SS. ANA positive 80%. Rheumatoid factor positive 60%. OCULAR TESTS: Schirmer test (<5mm/5min), Rose Bengal/lissamine green staining. ORAL TESTS: unstimulated salivary flow (<1.5mL/15min), salivary scintigraphy, sialography. LABIAL SALIVARY GLAND BIOPSY: gold standard—focal lymphocytic sialadenitis, focus score ≥1. ACR/EULAR classification criteria: weighted scoring. Differential: sicca from medications (anticholinergics), HCV, HIV, sarcoidosis, IgG4-related disease, graft-versus-host, prior head/neck radiation. Management: SYMPTOMATIC—SICCA: artificial tears (preservative-free), punctal plugs, cyclosporine ophthalmic emulsion, oral muscarinic agonists (pilocarpine, cevimeline) for dry mouth, salivary stimulants (sugar-free lozenges, gum), aggressive dental hygiene, fluoride, anticaries. ARTHRALGIA/FATIGUE: hydroxychloroquine (first-line). NSAIDs, low-dose glucocorticoids. SYSTEMIC ORGAN INVOLVEMENT: corticosteroids, immunosuppressants (mycophenolate, azathioprine, rituximab—refractory). LYMPHOMA: chemotherapy. Prognosis: excellent in primary SS without severe systemic involvement; increased lymphoma risk mandates vigilance. Zambian context: limited diagnostic tests (Schirmer strips accessible, autoantibodies limited); clinical diagnosis—sicca symptoms + anti-Ro/anti-La if available. Hydroxychloroquine accessible for arthralgia, fatigue. Vigilance for lymphoma: unexplained persistent parotid enlargement, lymphadenopathy.