Rheumatology

01

Approach to Rheumatologic Disease

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Inflammatory Arthritis

Morning stiffness >60 min · worse with rest, better with activity · night pain · swelling, erythema, warmth · constitutional symptoms (fever, fatigue, malaise) · ↑ESR/CRP · anemia of inflammation.

Synovial Fluid Analysis

Appearance · WBC · PMN proportion

Category	Appearance	WBC	PMN
Non-inflammatory	clear	`200–2,000`	<25%
Inflammatory	cloudy	`2,000–20,000`	>50%
Crystal-induced	cloudy	`10,000–50,000`	∼50%
Septic (bacterial)	cloudy / pus	`>50,000`	>75%
Septic (fungal / mycobacterial)	cloudy	`10,000–30,000`	—

Pattern of Joint Involvement

Number of joints · tempo

Pattern	Acute (<6 wk)	Chronic (≥6 wk)
Mono (1 joint)	infection, gout, pseudogout, trauma, hemarthrosis	OA, inflammatory, infection (fungal, TB, syphilis, Lyme), AVN
Oligo (2–4) / Poly (≥5)	viral, IE, Lyme, RF, disseminated gonococcal, HSV, HBV, parvovirus, HIV	RA, SLE, PsA, gout, pseudogout, reactive, paraneoplastic, OA

Seropositive: RA, SLE, SSc, Sjögren, IIM, MCTD
Seronegative: PsA, IBD-related, AS, reactive, undifferentiated
OA distribution: DIP, 1st CMC, spine, hips, knees, 1st MTP

Autoantibody Associations

Antibody → disease

Antibody	Association
ANA	SLE, SSc, Sjögren, MCTD
Anti-dsDNA	SLE
Anti-Sm	SLE
Anti–U1-RNP	MCTD, SLE
Anticentromere	Limited SSc
Anti–Scl-70	Diffuse SSc
Anti-Ro/SSA, Anti-La/SSB	Sjögren, SLE, RA, SSc, sicca, neonatal SLE
Antihistone	Drug-induced SLE
c-ANCA / anti-PR3	GPA
p-ANCA / anti-MPO	EGPA, MPA
Anti–Jo-1	Polymyositis, antisynthetase
Anti-CCP	RA
RF	RA, Sjögren, cryoglobulinemia

First-line imaging › X-ray
Bony erosions › CT
Acute synovitis › US

02

Rheumatoid Arthritis

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Clinical Picture

Symmetrical, small-joint polyarthritis — MCPs, PIPs, MTPs, wrists. Late deformities: swan-neck, Boutonnière, ulnar deviation, MCP subluxation. Risk factors: anti-CCP, smoking.

ACR/EULAR Classification — score ≥6 → RA

Sum across four domains

Domain	Criteria (points)
Joints	1 large (0) · 2–10 large (1) · 1–3 small (2) · 4–10 small (3) · >10 incl. ≥1 small (5)
Serology	RF & CCP −ive (0) · low +ive (2) · >3× ULN (3)
ESR/CRP	normal (0) · abnormal (1)
Duration	<6 wk (0) · ≥6 wk (1)

X-ray › periarticular osteopenia, joint-space narrowing, marginal erosions

Extra-articular Manifestations

Pericarditis, myocarditis, CAD, ILD, pleural effusion, pulmonary nodules, bronchiolitis obliterans, Caplan's syndrome, anemia, reactive thrombocytosis, Felty syndrome (seropositive RA + splenomegaly + neutropenia), lymphoproliferative disease, carpal tunnel, C1–C2 subluxation, rheumatoid nodules, vasculitis, keratoconjunctivitis sicca, scleritis, episcleritis, keratitis, Raynaud, Sweet syndrome, pyoderma gangrenosum, HF, osteoporosis, cricoarytenoid arthritis (hoarseness, sore throat, dysphagia, stridor).

Non-pharm › smoking cessation, calcium/vit D, regular DXA, vaccinations, PT/OT

DMARD Escalation

Assess response at 3–6 months

csDMARD — MTX / LEF / HCQ / SSZ

› switch csDMARD

› MTX + anti-TNF (adalimumab, infliximab, certolizumab, etanercept, golimumab)

› MTX + abatacept / RTX / tocilizumab / sarilumab / anakinra · or small molecule (tofacitinib, baricitinib, upadacitinib, apremilast)

› DMARD not previously trialed

HBcAb + HBsAb › prophylactic antiviral therapy
Bridging / symptomatic › NSAIDs, low-dose GC ≤3 mo
Low activity / remission ≥6 mo › reduce doses
Pregnancy › HCQ, SSZ, AZA, CNI (cyclosporine, tacrolimus), TNFi · stop MTX 3 mo before conception

DMARD Toxicity & Monitoring

Adverse effects · baseline · surveillance

Drug	Key toxicity	Monitoring
MTX	hepatotoxicity, nausea, pancytopenia, pneumonitis, pulmonary fibrosis, oral ulcers, alopecia, teratogenic, accelerated nodulosis › add folic acid. CI: pregnancy, breastfeeding, ILD, advanced liver disease, marrow failure, active severe infection	Baseline HCV/HBV, CBC, LFTs, Cr, CXR · CBC/LFTs/Cr 2–4 wk ×3 mo then annually
HCQ	retinal toxicity, rash, photosensitivity, myotoxicity, cardiotoxicity	Baseline fundoscopy, visual fields, OCT · annual screening after 5 y
LEF	nausea, abdominal pain, dyspepsia, diarrhea, hepatotoxicity, HTN, myelosuppression, peripheral neuropathy, teratogenic	Baseline CBC, LFTs, Cr, HBV, HCV · CBC/LFTs/Cr 2–4 wk ×3 mo then annually
SSZ	GI toxicity, headache, rash	Baseline CBC, LFTs, Cr · CBC/LFTs/Cr 2–4 wk ×3 mo then annually
Biologics	infection, drug-induced SLE, local skin reactions, malignancy, HF (TNFi). TNFi CI: HF NYHA III/IV, skin cancer, demyelinating disease, lymphoma ≤5 y	Baseline HBV, HCV, HIV, PPD, IGRA, CXR, CBC, LFTs, lipids
JAKi	MI, CVA, cancer, VTE, GI perforation	—

Tocilizumab/tofacitinib › recheck lipids after 3 mo
Latent TB › 1 month treatment · active TB › full treatment before biologic

03

Spondyloarthritis

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Clinical Picture

Asymmetrical, oligoarticular, larger joints, spinal involvement, DIPs, enthesitis (Achilles tendon, plantar fascia) — seronegative.

AxSpA criteria › sacroiliitis on imaging (XR or MRI) + ≥1 clinical SpA feature, or HLA-B27 + ≥2 clinical SpA features

Ankylosing Spondylitis

Sacroiliitis, ankylosis, age <45 y, ↓ lumbar movement, diminished chest expansion › HLA-B27.

First-line › NSAIDs, GC injection
High activity › TNFi, IL-17i (secukinumab, ixekizumab, bimekizumab) › JAKi (tofacitinib, upadacitinib)
New back pain or spinal trauma › CT/MRI spine

Psoriatic arthritis › psoriasis, peripheral arthritis, DIP, dactylitis, ↑ gout › MTX, biologics, JAKi. Explosive onset / severe flare › test HIV. XR hands › pencil-in-cup deformity
Reactive arthritis › 2–3 wk post GU/GI infection (Salmonella, Shigella, Campylobacter, Yersinia, Chlamydia); monoarthritis or acute asymmetric oligoarthritis, sacroiliitis, conjunctivitis, keratoderma blennorrhagicum, circinate balanitis, urethritis › NSAIDs, IA GC. STI › antibiotics · recurrent/chronic › SSZ
Enteropathic › IBD, AxSpA, sacroiliitis, peripheral arthritis, pyoderma gangrenosum › TNFi (infliximab), SSZ, AZA, MTX

Imaging & Extra-articular

MRI/XR SI & LS spine › sacroiliitis, erosions, pseudowidening, sclerosis, ankylosis, subchondral sclerosis, vertebral body squaring, bamboo spine.

Extra-articular › anterior uveitis, erythema nodosum, aortitis, aortic regurgitation, aortic aneurysm, cardiac conduction defects, apical pulmonary fibrosis, cauda equina syndrome.

Exercise, smoking cessation, PT, OT, vaccinations

04

Osteoarthritis

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Clinical Picture

Lower cervical/lumbar spine, hips, knees, DIP, PIP, 1st CMC. Dull pain, gradual onset, worse with activity, relieved with rest, morning stiffness <30 min, reduced motion, crepitus, tenderness, Heberden & Bouchard nodes, 1st CMC squaring.

Risk factors › obesity, female, older age, trauma, smoking, repetitive load
Erosive inflammatory OA › pain, soft-tissue swelling, PIP, DIP, flares · XR central interphalangeal erosions, "gull-wing" deformity
Secondary OA › previous injury, hemochromatosis (age <50 y, atypical joints)
XR › joint-space narrowing, subchondral sclerosis, osteophytes, subchondral cysts

Management

Conservative first; escalate by site & severity

Joint protection, exercise, weight loss, footwear, assistive devices

› topical / PO NSAIDs › duloxetine

› acute knee flare › IA GC

› persistent hip/knee pain or affected ADLs › joint arthroplasty

NSAID Contraindications

CAD · HF · CKD · PUD

05

Crystal Arthropathies

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Gout

Acute flares, monoarthritis, 1st MTP, knee, tophi › synovial fluid + blood cultures (r/o septic arthritis) › NSAIDs, colchicine, IA/PO GC.

RF › purine-rich diet (beer, red meat, seafood), CKD, thiazides, ASA, pyrazinamide, TLS, hemolysis, polycythemia, post-menopausal, obesity, cyclosporine, lead toxicity
Synovial fluid › monosodium urate, needle-shaped, −ively birefringent, WBC 2,000–100,000
XR › punched-out lesions, overhanging edges

Urate-Lowering Therapy

Indications (allopurinol, febuxostat) › ≥2 attacks/y, tophaceous gout, radiographic damage, recurrent uric-acid urolithiasis, ≥1 episode + RF (CKD 3/4, urate >535). Cover with colchicine/NSAID/low-dose GC ×3 mo.

Frequent flares + CI to standard therapy › anakinra, canakinumab
Allopurinol hypersensitivity (TEN/SJS, fever, eosinophilia, hepatic necrosis, AKI, diarrhea) › RF HLA-B5801 (SE/E Asian, African)
Recurrent/tophaceous gout › IV pegloticase + methotrexate (immunogenicity)
Target urate › <356 · <300 if tophi

Pseudogout (CPPD)

Acute, chronic, asymptomatic, OA, mono/oligoarthritis.

RF › hypothyroidism, hypomagnesemia, hypophosphatemia, hemochromatosis, hyperparathyroidism, Wilson disease
XR › chondrocalcinosis · Synovial fluid › rhomboid-shaped, +ively birefringent
BCP › calcific tendinitis (Milwaukee shoulder): older female, acute onset, destructive shoulder arthropathy
Crowned dens syndrome › acute/subacute upper neck pain, limited ROM, ↑ESR/CRP, fever
Acute › IA/PO GC, colchicine, NSAIDs
CPPD polyarthritis (wrists, MCPs) › low-dose colchicine, low-dose NSAIDs, low-dose GC

06

Systemic Lupus Erythematosus

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Clinical Picture — F:M 9:1

Malar rash (nasolabial sparing, photosensitivity), inflammatory arthritis, weight loss, fever, hair loss, anemia of inflammation, autoimmune hemolytic anemia, digit subluxation, swan-neck deformities, ulnar deviation, hip AVN (MRI), neuropsychiatric (headache, mild cognitive dysfunction, mood disorder), lupus pneumonitis, DAH, lupus nephritis, serositis, myocarditis, Libman–Sacks endocarditis, CAD. Lupus pneumonitis: cough, dyspnea, fever, hypoxemia, pleuritic pain, infiltrates.

ACR/EULAR Criteria

ANA ≥1:80 + ≥1 clinical criterion + ≥10 points

Domain	Features
Constitutional	fever `>38.3`
Hematologic	leukopenia, thrombocytopenia, autoimmune hemolysis
Neuropsychiatric	delirium, psychosis, seizure
Mucocutaneous	non-scarring alopecia, oral ulcers, subacute/discoid lupus, acute cutaneous lupus
Serosal	pleural/pericardial effusion, acute pericarditis
Renal	proteinuria, class II–IV lupus nephritis
Immunologic	APS Ab, low C3/C4, anti-dsDNA, anti-Smith

Disease activity › ↓C3/C4, ↑anti-dsDNA, ↑ESR › short-term GC
Anti-dsDNA › lupus nephritis
Anti-Ro (SSA) / anti-La (SSB) › congenital heart block, neonatal cutaneous lupus
Drug-induced lupus › arthritis, fever, serositis › ANA, anti-histone › stop causative drug (procainamide, hydralazine, isoniazid, TNFi, phenytoin, carbamazepine, minocycline)

Treatment Escalation

Avoid triggers; manage CVD risk

HCQ

› add MTX / AZA / MMF

› belimumab, anifrolumab

Flare › short-term PO/IV GC

Severe / organ-threatening (profound cytopenias, class III/IV nephritis, seizures, psychosis, RPGN) › high-dose GC, CYC, RTX, MMF

HCQ retinal toxicity › annual ophthalmologic exam
Avoid triggers › sunlight, infections, smoking, sulfa drugs, echinacea, alfalfa sprouts · sun protection, vaccinations, exercise, annual cervical cancer screening
Pregnancy › disease control ≥6 mo · HCQ, AZA, tacrolimus, GC

Lupus Nephritis

Biopsy class → therapy · target BP <130/80

Class	Lesion	Therapy
I / II	minimal / mesangial proliferative	HCQ + RAASi, ↓ Na intake
III / IV	focal & diffuse glomerular	GC + immunosuppressive (MMF, CYC, CNI, RTX, AZA)
V	membranous	RASi · proteinuria `>3.5 g/d` › GC + immunosuppressive
VI	advanced sclerosing	RRT

Hematuria, cellular casts, proteinuria >0.5 g/24h, spot UPCR >500 mg/g, ↓GFR › renal biopsy
↓eGFR › SGLT2i · APS nephropathy › warfarin

Antiphospholipid Syndrome

VTE, livedo reticularis, arterial thrombosis, skin necrosis, cardiac vegetation, pre-eclampsia, placental insufficiency, prolonged aPTT, cytopenias › 2 +ive Ab ≥12 wk apart (lupus anticoagulant, anti-cardiolipin, anti–β2-glycoprotein) › warfarin.

CAPS (≥3 organs ≤7 days, multi-organ failure) › therapeutic anticoagulation + pulse steroids + PLEX/IVIG › eculizumab/RTX/CYC
Obstetric › ASA + prophylactic LMWH through pregnancy until 6 wk postpartum
LA +ive without prior thrombosis › ASA

07

Sjögren Disease

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Clinical Picture

Primary or secondary (RA, SLE). Xerostomia, keratoconjunctivitis, salivary gland (parotid) enlargement. Extra-glandular › lymphadenopathy, arthritis, vasculitis, demyelinating neuropathy, GN, TIN › RTA, ↑ DLBCL, ↑ MALT lymphoma, ILD.

ACR/EULAR criteria › ocular/oral dryness score >4, minor salivary gland biopsy focus score >1 (focal lymphocytic sialadenitis), anti-SSA, Schirmer test <5 mm in ≥1 eye, unstimulated salivary flow <0.1 ml/min, ocular stain score >5
Labs › anti-Ro/SSA, anti-La/SSB, RF, ANA, hypergammaglobulinemia
Anti-Ro/SSA, anti-La/SSB › neonatal heart block › fetal echo

Management

Symptomatic first

Artificial tears, artificial saliva, mouth lubricants, sugar-free candy

› severe systemic manifestations › immunosuppressives

08

Systemic Sclerosis

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Clinical Picture

Raynaud, calcinosis, esophageal dysmotility, GERD, sclerodactyly, telangiectasias, digital pitting, small-bowel pseudo-obstruction, SIBO, malabsorption, inflammatory arthritis (MCP, PIP, wrists, ankles).

Subtypes

Skin distribution · antibody · organ risk

Type	Features	Antibody
Localized	morphea, linear scleroderma	—
Limited	distal limb skin thickening, PAH › PBC screening	ANA, anticentromere
Diffuse	proximal limb/torso thickening, ILD (NSIP, UIP), SRC, GAVE	ANA, anti–Scl-70

Scleroderma renal crisis › AKI, HTN, MAHA, proteinuria, thrombocytopenia › anti-RNA pol III › ACEi (captopril) · ↑ risk with prednisone
GAVE (gastric antral vascular ectasia) › UGIB, chronic anemia › anti-RNA pol III › EGD
Surveillance › baseline HRCT, PFT, NT-proBNP, echo · then annual NT-proBNP, echo, PFT
Esophageal/intestinal dysmotility › metoclopramide · ILD › MMF
Pregnancy CI › PAH, severe ILD

Raynaud Phenomenon

Triphasic discoloration (pallor → cyanosis → erythema), cold-triggered › nailfold capillary exam › avoid cold, smoking cessation, CCB (amlodipine, felodipine, nifedipine), sildenafil, topical nitroglycerin.

Primary › symmetric, ANA −ive, female, 20s
Secondary › asymmetric, progressive, nailfold capillary destruction, dilated loops, digit ulceration, thumb involvement, male, age >40 y (SSc, MCTD, SLE, hypothyroidism, carcinoid, HBV, HCV, parvovirus B19, hematologic malignancy)

09

Idiopathic Inflammatory Myopathies

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DM / PM

Symmetrical muscle weakness (insidious, proximal > distal; heart, diaphragm, oropharynx, esophagus), myocarditis, arrhythmias, CHF, ILD (NSIP, UIP), PAH, Gottron papules, shawl sign, heliotrope rash, generalized erythroderma, periungual erythema, mechanic's hands, scalp psoriasiform changes, calcinosis cutis › anti-Jo-1, anti-Mi2 (DM).

Workup › ↑CK, ↑ALT/AST, ↑aldolase, anti-Jo1, anti-MDA5, anti-NXP2, anti-TIF1-γ, ANA, muscle biopsy, EMG, ECG, echo, SLP, spirometry
Normal CK › amyopathic DM · muscle MRI › edema
Antisynthetase syndrome › ILD, inflammatory polyarthritis, fever, Raynaud, mechanic's hands › anti-Jo1
Anti-MDA5 › rapidly progressive ILD, skin ulceration
Malignancy-associated › anti-NXP2, anti-TIF1-γ
IBM › older male, insidious, distal > proximal, asymmetric, dysphagia, atrophy, facial weakness, poor treatment response › exercise, PT
IMNM/NAM › severe proximal myopathy, markedly ↑CK, no skin findings › anti-HMG CoA reductase · biopsy › necrosis
Malignancy screening (ovarian, lung, breast, pancreas, stomach, colon, lymphoma) › DM > PM/IBM

Treatment

GC › prophylactic calcium, vit D, bisphosphonates

High-dose PO GC

› DMARD (MTX, AZA, tacrolimus, MMF)

Skin › HCQ

Refractory / severe › IVIG, RTX, CTX, abatacept

Rapidly progressive ILD › IV GC + 2 immunosuppressives (RTX, CYC, IVIG, CNI, MMF)

10

Systemic Vasculitis

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Classification by Vessel Size

Caliber → entities

Vessel	Entities
Capillary	Goodpasture syndrome
Small (immune complex)	IgA, cryoglobulinemia, leukocytoclastic
Small (ANCA)	MPA, GPA, EGPA
Small (secondary)	HBV, HCV, syphilis, COVID, levamisole, SLE, RA, Sjögren, sarcoidosis, malignancy
Medium	PAN, Kawasaki, primary CNS angiitis
Large	Takayasu, GCA
Variable	Behçet, Cogan (keratitis, sensorineural deafness), thromboangiitis obliterans

Goodpasture › pulmonary-renal syndrome › anti-GBM › PLEX
IgA (HSP) › preceding URTI, palpable purpura, MSK, GI, GN › skin/renal biopsy (IgA deposition) › self-limiting · severe/persistent GN › GC or CYC
Leukocytoclastic (hypersensitivity) › palpable purpura, vesicles, pustules, maculopapular lesions, urticaria (viral, drugs, malignancy) › skin biopsy (neutrophilic infiltrate, leukocytoclasia, fibrinoid necrosis) › stop causative drug · persistent › topical/low-dose PO GC, dapsone, colchicine

Cryoglobulinemia

Skin lesions (red macules, palpable purpura, nodules, ulcers, acrocyanosis, livedo), mononeuritis multiplex, arthralgias, GN, hepatitis, CNS, mesenteric ischemia, DAH › cryoglobulins, HCV, HIV, ANA, anti-Ro/La, RF, ↓C3/C4.

Type I (vascular occlusion) › MGUS, MM, CLL, WM › treat underlying ± PLEX
Type II/III (immune complex) › HCV, HBV, HIV, IE, Sjögren, lymphoproliferative › GC ± colchicine · HCV › antiviral
Severe › GC + RTX/CYC + PLEX

ANCA-Associated Vasculitis

Distinguishing features · ANCA

Type	Features	ANCA
MPA	pulmonary infiltrates, palpable purpura, RPGN, peripheral neuropathy	p-ANCA
GPA	recurrent otitis media, rhinosinusitis, saddle-nose, tracheal collapse, pulmonary infiltrates/cavities/hemoptysis, pauci-immune GN, epistaxis, nasal ulcers	c-ANCA (PR3)
EGPA	asthma, allergic rhinitis, eosinophilia, ↑IgE, myocarditis, sinusitis, infiltrates/hemoptysis, purpura, GI, neuropathy	p-ANCA (MPO)

GPA › GC + RTX/MTX/AZA/MMF · severe (GN, DAH, CNS, retro-orbital, cardiac, mononeuritis, mesenteric) › pulse IV GC + RTX/CYC · maintenance › RTX/AZA. MPA managed as GPA
EGPA › GC + MEP, GC + MTX/AZA/MMF or RTX · severe (proteinuria >1 g/24h, Cr >138, GI, cardiac, CNS) › pulse IV GC + RTX/CYC

Medium-Vessel

PAN › mesenteric arteritis, mononeuritis multiplex, constitutional, orchitis, cutaneous (deep ulcers, livedo, purpura), myalgia, arthritis, renal impairment › HBV, HCV, biopsy › GC + MTX/AZA · MRA mesenteric/renal › aneurysms, stenoses · severe › high-dose IV GC + CYC
Kawasaki › children, conjunctivitis, cervical lymphadenopathy, strawberry tongue, rash, coronary artery aneurysm
Primary CNS angiitis › recurrent headaches, CVA/TIA, progressive encephalopathy › LP, MRI/MRA, brain biopsy (granulomatous vasculitis) › high-dose GC + CYC

Large-Vessel

Takayasu › aortitis, pulseless peripheries, Asian female age <40 y, HTN, >20 SBP arm difference, angina, constitutional, bruits, claudication › MRA/CTA › prednisone, MTX/AZA · critical stenosis › bypass/angioplasty
GCA › headache, jaw claudication, diplopia, amaurosis fugax, scalp tenderness, posterior CVA, constitutional, tender/absent temporal artery pulses, PMR (50%) › ↑ESR, US temporal arteries (halo sign) › high-dose GC + TCZ/MTX › temporal artery biopsy (≤1 mo steroids) › CTA/MRA neck + chest/abdo/pelvis. Visual symptoms/CVA › IV pulse GC ×3 d then prednisone 1 mg/kg + TCZ

PMR › bilateral shoulder/hip aching + morning stiffness >45 min, GCA (15%) › PO prednisone 12.5–20 mg/d then taper · US bursitis/tenosynovitis/synovitis · normal RF, anti-CCP, CK · refractory › sarilumab
Behçet › oral/genital ulcers, uveitis, asymmetric oligoarthritis, CNS, VTE, pulmonary/peripheral aneurysms › HLA-B51, +ive pathergy test › prednisone · ulcers › topical GC, colchicine, apremilast, AZA, TNFi · aneurysms › surgery, CYC, infliximab · major (uveitis, CNS, GI, large artery) › AZA/CYC/MMF
Thromboangiitis obliterans › male smokers, necrotic digit tips, hand/foot claudication

11

Misc. Systemic Inflammatory Disease

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Relapsing Polychondritis

Rare; recurrent cartilage inflammation (nasal bridge, ear, bronchi), auricular chondritis, hearing loss, saddle-nose, subglottic/tracheal stenosis, episcleritis, scleritis, asymmetric inflammatory arthritis, seronegative › tissue biopsy, HLA-DR4, CT neck/chest, PFT, tracheobronchoscopy.

Mild › NSAIDs, dapsone · acute/severe › high-dose GC ± MTX, infliximab, adalimumab, tocilizumab · airway stenosis › surgery

Autoinflammatory Disorders

Adult-onset Still disease › young female, fever >39 (evening spikes), fatigue, asymmetric oligoarthritis, myalgia, transient salmon-pink rash, sore throat, lymphadenopathy, hepatosplenomegaly, proteinuria, serositis, abnormal LFTs, ↑ESR/CRP, ferritin >2500, neutrophilic leukocytosis › IL1i (anakinra, canakinumab) or IL6i (tocilizumab) · high activity › high-dose GC + IL1i/IL6i
MAS (secondary HLH) › persistent fever, splenomegaly, ferritin >10,000, cytopenias, abnormal LFTs, coagulopathy, hypertriglyceridemia › high-dose GC + IL1i, CNI, IFN-γi, IVIG
FMF › autosomal recessive, age <20 y, abdominal pain, serositis, flares 12–72 h, LL monoarthritis, LL erysipeloid rash, ↑ amyloidosis › colchicine
VEXAS › X-linked male age >50 y, fever, cytopenias, ocular, leukocytoclastic vasculitis, auricular/nasal chondritis, serositis, alveolitis, macrocytosis, anemia, thrombocytopenia, lymphopenia, ↑ VTE › genetic testing (UBA1) › high-dose GC + immunosuppressive (JAKi, IL6i). Associations: MDS, MM, MGUS · BMB › vacuoles in myeloid/erythroid precursors, dysplastic marrow

Heritable Connective Tissue

EDS › joint laxity, instability, dislocations, MSK pain, early OA, easy bruising, mild skin laxity, mitral/tricuspid prolapse, aortic root dilation
Marfan › hypermobility, tall stature, dolichostenomelia, arachnodactyly, pectus excavatum/carinatum, scoliosis, hyperextensible skin, myopia, ectopia lentis, high-arched palate, pneumothorax, blue sclerae › BBs
Osteogenesis imperfecta › fractures, short stature, dentinogenesis imperfecta, hearing loss, blue sclerae › bisphosphonates

IgG4-Related Disease

Fibro-inflammatory lesions of salivary glands, pancreas, lungs, kidneys, aorta, retroperitoneum › ↑IgG4, biopsy (lymphoplasmacytic infiltrate, storiform fibrosis, IgG4 cells) › moderate/high-dose GC, AZA/RTX.

12

Fibromyalgia

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Clinical Picture

Widespread pain ≥3 months, sleep disturbance, fatigue, cognitive dysfunction, other causes excluded. Associations: Sjögren, SLE, RA.

Workup › CBC, RFT, TSH, ESR/CRP — normal
Non-pharm › exercise, CBT, sleep hygiene
Pharm › pregabalin, gabapentin, duloxetine, milnacipran
With depression › duloxetine, milnacipran, TCAs

13

MCTD & UCTD

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Mixed Connective Tissue Disease

Features of ≥2 of SLE, SSc, IIM, RA › anti–U1-RNP.

Raynaud, hand edema, sclerodactyly, inflammatory arthritis, synovitis, myositis, pericarditis, PAH, ILD, ↑ CVD

A study guide to rheumatology.

Approach to Rheumatologic Disease

Synovial Fluid Analysis

Pattern of Joint Involvement

Autoantibody Associations

Rheumatoid Arthritis

ACR/EULAR Classification — score ≥6 → RA

DMARD Toxicity & Monitoring

Spondyloarthritis

Osteoarthritis

Crystal Arthropathies

Systemic Lupus Erythematosus

ACR/EULAR Criteria

Lupus Nephritis

Sjögren Disease

Systemic Sclerosis

Subtypes

Idiopathic Inflammatory Myopathies

Systemic Vasculitis

Classification by Vessel Size

ANCA-Associated Vasculitis

Misc. Systemic Inflammatory Disease

Fibromyalgia

MCTD & UCTD

No Stalking
Allowed!

This Is Not
A Café.